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Surgery_Schwartz_10302 | Surgery_Schwartz | and meta-analysis examining the impact of incision on outcomes after abdominal surgery. Am J Surg. 2013;206:400-409. doi: 10.1016/j.amjsurg.2012.11.008Bilsel Y, Abci I. The search for ideal hernia repair; mesh materi-als and types. Int J Surg. 2012;10:317-321. doi: 10.1016/j.ijsu.2012.05.002Brown SR, Tiernan J. Transverse verses midline incisions for abdom-inal surgery. Cochrane Database Syst Rev. 2005;(4):CD005199. doi: 10.1002/14651858.CD005199.pub2Caro-Tarrago A, Olona Casas C, Jimenez Salido A, et al. Prevention of incisional hernia in midline laparotomy with an onlay mesh: a randomized clinical trial. World J Surg. 2014;38:2223-2230. doi: 10.1007/s00268-014-2510-6Conze J, Kingsnorth AN, Flament JB, et al. Randomized clinical trial comparing lightweight composite mesh with polyester or polypropylene mesh for incisional hernia repair. Br J Surg. 2005;92:1488-1493. doi: 10.1002/bjs.5208de Vries Reilingh TS, van Goor H, Rosman C, et al. “Compo-nents separation technique” for the | Surgery_Schwartz. and meta-analysis examining the impact of incision on outcomes after abdominal surgery. Am J Surg. 2013;206:400-409. doi: 10.1016/j.amjsurg.2012.11.008Bilsel Y, Abci I. The search for ideal hernia repair; mesh materi-als and types. Int J Surg. 2012;10:317-321. doi: 10.1016/j.ijsu.2012.05.002Brown SR, Tiernan J. Transverse verses midline incisions for abdom-inal surgery. Cochrane Database Syst Rev. 2005;(4):CD005199. doi: 10.1002/14651858.CD005199.pub2Caro-Tarrago A, Olona Casas C, Jimenez Salido A, et al. Prevention of incisional hernia in midline laparotomy with an onlay mesh: a randomized clinical trial. World J Surg. 2014;38:2223-2230. doi: 10.1007/s00268-014-2510-6Conze J, Kingsnorth AN, Flament JB, et al. Randomized clinical trial comparing lightweight composite mesh with polyester or polypropylene mesh for incisional hernia repair. Br J Surg. 2005;92:1488-1493. doi: 10.1002/bjs.5208de Vries Reilingh TS, van Goor H, Rosman C, et al. “Compo-nents separation technique” for the |
Surgery_Schwartz_10303 | Surgery_Schwartz | or polypropylene mesh for incisional hernia repair. Br J Surg. 2005;92:1488-1493. doi: 10.1002/bjs.5208de Vries Reilingh TS, van Goor H, Rosman C, et al. “Compo-nents separation technique” for the repair of large abdominal wall hernias. J Am Coll Surg. 2003;196:32-37. doi: 10.1016/S1072-7515(02)01478-3Deerenberg EB, Harlaar JJ, Steyerberg EW, et al. Small bites versus large bites for closure of abdominal midline incisions (STITCH): a double-blind, multicentre, randomised con-trolled trial. Lancet. 2015;386(10000):1254-1260. doi: http://dx.doi.org/10.1016/S0140-6736(15)60459-7DeMaria EJ, Moss JM, Sugerman HJ. Laparoscopic intraperito-neal polytetrafluoroethylene (PTFE) prosthetic patch repair of ventral hernia. Surg Endosc. 2000;14:326-329. doi: 10.1007/s004640020013den Hartog D, Dur AHM, Tuinebreijer WE, Kreis RW. Open surgi-cal procedures for incisional hernias. Cochrane Database Syst Rev. 2008; doi: 10.1002/14651858.CD006438.pub2Ellis H. Applied anatomy of abdominal incisions. Br J | Surgery_Schwartz. or polypropylene mesh for incisional hernia repair. Br J Surg. 2005;92:1488-1493. doi: 10.1002/bjs.5208de Vries Reilingh TS, van Goor H, Rosman C, et al. “Compo-nents separation technique” for the repair of large abdominal wall hernias. J Am Coll Surg. 2003;196:32-37. doi: 10.1016/S1072-7515(02)01478-3Deerenberg EB, Harlaar JJ, Steyerberg EW, et al. Small bites versus large bites for closure of abdominal midline incisions (STITCH): a double-blind, multicentre, randomised con-trolled trial. Lancet. 2015;386(10000):1254-1260. doi: http://dx.doi.org/10.1016/S0140-6736(15)60459-7DeMaria EJ, Moss JM, Sugerman HJ. Laparoscopic intraperito-neal polytetrafluoroethylene (PTFE) prosthetic patch repair of ventral hernia. Surg Endosc. 2000;14:326-329. doi: 10.1007/s004640020013den Hartog D, Dur AHM, Tuinebreijer WE, Kreis RW. Open surgi-cal procedures for incisional hernias. Cochrane Database Syst Rev. 2008; doi: 10.1002/14651858.CD006438.pub2Ellis H. Applied anatomy of abdominal incisions. Br J |
Surgery_Schwartz_10304 | Surgery_Schwartz | Tuinebreijer WE, Kreis RW. Open surgi-cal procedures for incisional hernias. Cochrane Database Syst Rev. 2008; doi: 10.1002/14651858.CD006438.pub2Ellis H. Applied anatomy of abdominal incisions. Br J Hosp Med. 2010;71:M36-M37. doi: 10.12968/hmed.2010.71.Sup3.46991Franz MG. The biology of hernia formation. Surg Clin North Am. 2008;88:1-15.García CM, Ruiz SG, Franco CC. Anatomy of the abdominal wall. In: Suárez Grau JM, Bellido Luque JA, eds. Advances in Lap-aroscopy of the Abdominal Wall Hernia. London: Springer; 2014:7-22.Halm JA, Heisterkamp J, Veen HF, Weidema WF. Long-term follow-up after umbilical hernia repair: are there risk factors for recurrence after simple and mesh repair. Hernia. 2005;9: 334-337. doi: 10.1007/s10029-005-0010-1Hanson J. Netter’s Clinical Anatomy. 3rd ed. Philadelphia: Elsevier; 2014.Hasson HM. A modified instrument and method for laparoscopy. Am J Obset Gynecol. 1971;110:886-887. doi: 10.1016/0002-9378(71)90593-XHegazy AA. Anatomy and embryology of | Surgery_Schwartz. Tuinebreijer WE, Kreis RW. Open surgi-cal procedures for incisional hernias. Cochrane Database Syst Rev. 2008; doi: 10.1002/14651858.CD006438.pub2Ellis H. Applied anatomy of abdominal incisions. Br J Hosp Med. 2010;71:M36-M37. doi: 10.12968/hmed.2010.71.Sup3.46991Franz MG. The biology of hernia formation. Surg Clin North Am. 2008;88:1-15.García CM, Ruiz SG, Franco CC. Anatomy of the abdominal wall. In: Suárez Grau JM, Bellido Luque JA, eds. Advances in Lap-aroscopy of the Abdominal Wall Hernia. London: Springer; 2014:7-22.Halm JA, Heisterkamp J, Veen HF, Weidema WF. Long-term follow-up after umbilical hernia repair: are there risk factors for recurrence after simple and mesh repair. Hernia. 2005;9: 334-337. doi: 10.1007/s10029-005-0010-1Hanson J. Netter’s Clinical Anatomy. 3rd ed. Philadelphia: Elsevier; 2014.Hasson HM. A modified instrument and method for laparoscopy. Am J Obset Gynecol. 1971;110:886-887. doi: 10.1016/0002-9378(71)90593-XHegazy AA. Anatomy and embryology of |
Surgery_Schwartz_10305 | Surgery_Schwartz | Philadelphia: Elsevier; 2014.Hasson HM. A modified instrument and method for laparoscopy. Am J Obset Gynecol. 1971;110:886-887. doi: 10.1016/0002-9378(71)90593-XHegazy AA. Anatomy and embryology of umbilicus in newborns: a review and clinical correlations. Frontiers Med. 2016;10: 271-277. doi: 10.1007/s11684-016-0457-8Henrich K, Huemmer HP, Reingruber B, Weber PG. Gastroschisis and omphalocele: treatments and long-term outcomes. Ped Surg Int. 2008;24:167-173. doi: 10.1007/s00383-007-2055-yHoward JH, Pollock R. Intra-abdominal and abdominal wall des-moid fibromatosis. Oncol Ther. 2016;4:57-72.Inaba T, Okinaga K, Fukushima R, et al. Prospective randomized study of two laparotomy incisions for gastrectomy: midline incision versus transverse incision. Gastric Cancer. 2004;7: 167-171. doi: 10.1007/s10120-004-0291-6Le Huu Nho R, Mege D, Ouaïssi M, et al. Incidence and prevention of ventral incisional hernia. J Visc Surg. 2012;149:e3-e14.Lowe JB, Garza JR, Bowman JL, Rohrich RJ, Strodel | Surgery_Schwartz. Philadelphia: Elsevier; 2014.Hasson HM. A modified instrument and method for laparoscopy. Am J Obset Gynecol. 1971;110:886-887. doi: 10.1016/0002-9378(71)90593-XHegazy AA. Anatomy and embryology of umbilicus in newborns: a review and clinical correlations. Frontiers Med. 2016;10: 271-277. doi: 10.1007/s11684-016-0457-8Henrich K, Huemmer HP, Reingruber B, Weber PG. Gastroschisis and omphalocele: treatments and long-term outcomes. Ped Surg Int. 2008;24:167-173. doi: 10.1007/s00383-007-2055-yHoward JH, Pollock R. Intra-abdominal and abdominal wall des-moid fibromatosis. Oncol Ther. 2016;4:57-72.Inaba T, Okinaga K, Fukushima R, et al. Prospective randomized study of two laparotomy incisions for gastrectomy: midline incision versus transverse incision. Gastric Cancer. 2004;7: 167-171. doi: 10.1007/s10120-004-0291-6Le Huu Nho R, Mege D, Ouaïssi M, et al. Incidence and prevention of ventral incisional hernia. J Visc Surg. 2012;149:e3-e14.Lowe JB, Garza JR, Bowman JL, Rohrich RJ, Strodel |
Surgery_Schwartz_10306 | Surgery_Schwartz | 10.1007/s10120-004-0291-6Le Huu Nho R, Mege D, Ouaïssi M, et al. Incidence and prevention of ventral incisional hernia. J Visc Surg. 2012;149:e3-e14.Lowe JB, Garza JR, Bowman JL, Rohrich RJ, Strodel WE. Endoscopically assisted “components separation” for closure of abdominal wall defects. Plast Reconstr Surg. 2000; 105(2):720-729.Luijendijk RW, Hop WCJ, van den Tol MP, et al. A compari-son of suture repair with mesh repair for incisional her-nia. N Engl J Med. 2000;343:392-398. doi: 10.1056/NEJM200008103430603Maingot R, Zinner M, Ashley SW. Maingot’s Abdominal Opera-tions. New York: McGraw-Hill Medical; 2013.8Brunicardi_Ch35_p1549-p1566.indd 156312/02/19 9:59 AM 1564SPECIFIC CONSIDERATIONSPART IIMcBurney C. IV. The incision made in the abdominal wall in cases of appendicitis, with a description of a new method of operat-ing. Ann Surg. 1894;20:38-43.Mekonen HK, Hikspoors JPJM, Mommen G, et al. Develop-ment of the ventral body wall in the human embryo. J Anat. 2015;227:673-685. doi: | Surgery_Schwartz. 10.1007/s10120-004-0291-6Le Huu Nho R, Mege D, Ouaïssi M, et al. Incidence and prevention of ventral incisional hernia. J Visc Surg. 2012;149:e3-e14.Lowe JB, Garza JR, Bowman JL, Rohrich RJ, Strodel WE. Endoscopically assisted “components separation” for closure of abdominal wall defects. Plast Reconstr Surg. 2000; 105(2):720-729.Luijendijk RW, Hop WCJ, van den Tol MP, et al. A compari-son of suture repair with mesh repair for incisional her-nia. N Engl J Med. 2000;343:392-398. doi: 10.1056/NEJM200008103430603Maingot R, Zinner M, Ashley SW. Maingot’s Abdominal Opera-tions. New York: McGraw-Hill Medical; 2013.8Brunicardi_Ch35_p1549-p1566.indd 156312/02/19 9:59 AM 1564SPECIFIC CONSIDERATIONSPART IIMcBurney C. IV. The incision made in the abdominal wall in cases of appendicitis, with a description of a new method of operat-ing. Ann Surg. 1894;20:38-43.Mekonen HK, Hikspoors JPJM, Mommen G, et al. Develop-ment of the ventral body wall in the human embryo. J Anat. 2015;227:673-685. doi: |
Surgery_Schwartz_10307 | Surgery_Schwartz | of a new method of operat-ing. Ann Surg. 1894;20:38-43.Mekonen HK, Hikspoors JPJM, Mommen G, et al. Develop-ment of the ventral body wall in the human embryo. J Anat. 2015;227:673-685. doi: 10.1111/joa.12380Millbourn D, Cengiz Y, Israelsson L. Effect of stitch length on wound complications after closure of mid-line incisions. Arch Surg. 2009;144(11):1056-1059.Murphy KP, O’Connor OJ, Maher MM. Adult abdominal her-nias. Am J Roentgenol. 2014;202:W506-W511. doi: 10.2214/AJR.13.12071Mwachaka PM, Saidi HS, Odula PO, et al. Locating the arcuate line of Douglas: is it of surgical relevance? Clin Anat. 2010;23: 84-86. doi: 10.1002/ca.20877Nachiappan S, Markar S, Karthikesaligam A, et al. Prophylactic mesh placement in high-risk patients undergoing elective lapa-rotomy: a systematic review. World J Surg. 2013;37:1861-1871. doi: 10.1007/s00268-013-2046-1Palmer R. Safety in laparoscopy. J Reprod Med. 1974;13:1-5.Ponten JEH, Somers KYA, Nienhuijs SW. Pathogenesis of the epigastric hernia. Hernia. | Surgery_Schwartz. of a new method of operat-ing. Ann Surg. 1894;20:38-43.Mekonen HK, Hikspoors JPJM, Mommen G, et al. Develop-ment of the ventral body wall in the human embryo. J Anat. 2015;227:673-685. doi: 10.1111/joa.12380Millbourn D, Cengiz Y, Israelsson L. Effect of stitch length on wound complications after closure of mid-line incisions. Arch Surg. 2009;144(11):1056-1059.Murphy KP, O’Connor OJ, Maher MM. Adult abdominal her-nias. Am J Roentgenol. 2014;202:W506-W511. doi: 10.2214/AJR.13.12071Mwachaka PM, Saidi HS, Odula PO, et al. Locating the arcuate line of Douglas: is it of surgical relevance? Clin Anat. 2010;23: 84-86. doi: 10.1002/ca.20877Nachiappan S, Markar S, Karthikesaligam A, et al. Prophylactic mesh placement in high-risk patients undergoing elective lapa-rotomy: a systematic review. World J Surg. 2013;37:1861-1871. doi: 10.1007/s00268-013-2046-1Palmer R. Safety in laparoscopy. J Reprod Med. 1974;13:1-5.Ponten JEH, Somers KYA, Nienhuijs SW. Pathogenesis of the epigastric hernia. Hernia. |
Surgery_Schwartz_10308 | Surgery_Schwartz | Surg. 2013;37:1861-1871. doi: 10.1007/s00268-013-2046-1Palmer R. Safety in laparoscopy. J Reprod Med. 1974;13:1-5.Ponten JEH, Somers KYA, Nienhuijs SW. Pathogenesis of the epigastric hernia. Hernia. 2012;16:627-633. doi: 10.1007/s10029-012-0964-8Ramirez O, Ruas E, Dellon A. “Components separation” method for closure for abdominal-wall defects: an anatomic and clinical study. Plast Reconstr Surg. 1990;86(3):519-526.Rastegarpour A, Cheung M, Vardhan M, et al. Surgical mesh for ventral incisional hernia repairs: understanding mesh design. Plast Surg. 2016;24(1):41-50.Reilingh TS de V, van Goor H, Charbon JA, et al. Repair of giant midline abdominal wall hernias: “components separation tech-nique” versus prosthetic repair. World J Surg. 2007;31:756-763. doi: 10.1007/s00268-006-0502-xRozen WM, Ashton MW, Taylor GI. Reviewing the vascular supply of the anterior abdominal wall: redefining anatomy for increas-ingly refined surgery. Clin Anat. 2008;21:89-98. doi: 10.1002/ca.20585Salameh JR. | Surgery_Schwartz. Surg. 2013;37:1861-1871. doi: 10.1007/s00268-013-2046-1Palmer R. Safety in laparoscopy. J Reprod Med. 1974;13:1-5.Ponten JEH, Somers KYA, Nienhuijs SW. Pathogenesis of the epigastric hernia. Hernia. 2012;16:627-633. doi: 10.1007/s10029-012-0964-8Ramirez O, Ruas E, Dellon A. “Components separation” method for closure for abdominal-wall defects: an anatomic and clinical study. Plast Reconstr Surg. 1990;86(3):519-526.Rastegarpour A, Cheung M, Vardhan M, et al. Surgical mesh for ventral incisional hernia repairs: understanding mesh design. Plast Surg. 2016;24(1):41-50.Reilingh TS de V, van Goor H, Charbon JA, et al. Repair of giant midline abdominal wall hernias: “components separation tech-nique” versus prosthetic repair. World J Surg. 2007;31:756-763. doi: 10.1007/s00268-006-0502-xRozen WM, Ashton MW, Taylor GI. Reviewing the vascular supply of the anterior abdominal wall: redefining anatomy for increas-ingly refined surgery. Clin Anat. 2008;21:89-98. doi: 10.1002/ca.20585Salameh JR. |
Surgery_Schwartz_10309 | Surgery_Schwartz | Ashton MW, Taylor GI. Reviewing the vascular supply of the anterior abdominal wall: redefining anatomy for increas-ingly refined surgery. Clin Anat. 2008;21:89-98. doi: 10.1002/ca.20585Salameh JR. Primary and unusual abdominal wall hernias. Surg Clin North Am. 2008;88:45-60. doi: 10.1016/j.suc.2007.10.004Sanders DL, Kingsnorth AN. The modern management of inci-sional hernias. BMJ. 2012;344:e2843. doi: 10.1136/bmj.e2843Seiler CM, Deckert A, Diener MK, et al. Midline versus trans-verse incision in major abdominal surgery: a randomized, double-blind equivalence trial (POVATI: ISRCTN60734227). Ann Surg. 2009;249(6):913-920.Skandalakis P, Odyseas Z, Skandalakis J, Petros M. Spigelian her-nia: surgical anatomy, embryology, and technique of repair. Am Surg. 2006;72:42-48.Timmermans L, de Goede B, van Dijk SM, et al. Meta-analysis of sublay versus onlay mesh repair in incisional hernia surgery. Am J Surg. 2014;207:980-988. doi: 10.1016/j.amjsurg.2013.08.030Wilder F, D’Angelo S, Crago AM. Soft | Surgery_Schwartz. Ashton MW, Taylor GI. Reviewing the vascular supply of the anterior abdominal wall: redefining anatomy for increas-ingly refined surgery. Clin Anat. 2008;21:89-98. doi: 10.1002/ca.20585Salameh JR. Primary and unusual abdominal wall hernias. Surg Clin North Am. 2008;88:45-60. doi: 10.1016/j.suc.2007.10.004Sanders DL, Kingsnorth AN. The modern management of inci-sional hernias. BMJ. 2012;344:e2843. doi: 10.1136/bmj.e2843Seiler CM, Deckert A, Diener MK, et al. Midline versus trans-verse incision in major abdominal surgery: a randomized, double-blind equivalence trial (POVATI: ISRCTN60734227). Ann Surg. 2009;249(6):913-920.Skandalakis P, Odyseas Z, Skandalakis J, Petros M. Spigelian her-nia: surgical anatomy, embryology, and technique of repair. Am Surg. 2006;72:42-48.Timmermans L, de Goede B, van Dijk SM, et al. Meta-analysis of sublay versus onlay mesh repair in incisional hernia surgery. Am J Surg. 2014;207:980-988. doi: 10.1016/j.amjsurg.2013.08.030Wilder F, D’Angelo S, Crago AM. Soft |
Surgery_Schwartz_10310 | Surgery_Schwartz | van Dijk SM, et al. Meta-analysis of sublay versus onlay mesh repair in incisional hernia surgery. Am J Surg. 2014;207:980-988. doi: 10.1016/j.amjsurg.2013.08.030Wilder F, D’Angelo S, Crago AM. Soft tissue tumors of the trunk: management of local disease in the breast and chest and abdom-inal walls. J Surg Oncol. 2015;111:546-552. doi: 10.1002/jso.23843Zhang Y, Zhou H, Chai Y, Cao C, Jin K, Hu Z. Laparoscopic versus open incisional and ventral hernia repair: a systematic review and meta-analysis. World J Surg. 2014;38(9):2233-2240. doi: 10.1007/s00268-014-2578-zOmentumAbdulAziz A, El Zalabany T, Al Sayed AR, Al Ansari A. Idiopathic omental infarction, diagnosed and managed laparoscopi-cally: a case report. Case Rep Surg. 2013;2013:193546. doi: 10.1155/2013/193546Anyfantakis D, Kastanakis M, Petrakis G, et al. Rectus sheath hematoma in a single secondary care institution: a retrospective study. Hernia. 2015;19(3):509-512.Collins D, Hogan AM, O’Shea D, Winter DC. The omentum: | Surgery_Schwartz. van Dijk SM, et al. Meta-analysis of sublay versus onlay mesh repair in incisional hernia surgery. Am J Surg. 2014;207:980-988. doi: 10.1016/j.amjsurg.2013.08.030Wilder F, D’Angelo S, Crago AM. Soft tissue tumors of the trunk: management of local disease in the breast and chest and abdom-inal walls. J Surg Oncol. 2015;111:546-552. doi: 10.1002/jso.23843Zhang Y, Zhou H, Chai Y, Cao C, Jin K, Hu Z. Laparoscopic versus open incisional and ventral hernia repair: a systematic review and meta-analysis. World J Surg. 2014;38(9):2233-2240. doi: 10.1007/s00268-014-2578-zOmentumAbdulAziz A, El Zalabany T, Al Sayed AR, Al Ansari A. Idiopathic omental infarction, diagnosed and managed laparoscopi-cally: a case report. Case Rep Surg. 2013;2013:193546. doi: 10.1155/2013/193546Anyfantakis D, Kastanakis M, Petrakis G, et al. Rectus sheath hematoma in a single secondary care institution: a retrospective study. Hernia. 2015;19(3):509-512.Collins D, Hogan AM, O’Shea D, Winter DC. The omentum: |
Surgery_Schwartz_10311 | Surgery_Schwartz | M, Petrakis G, et al. Rectus sheath hematoma in a single secondary care institution: a retrospective study. Hernia. 2015;19(3):509-512.Collins D, Hogan AM, O’Shea D, Winter DC. The omentum: ana-tomical, metabolic, and surgical aspects. J Gastrointest Surg. 2009;13:1138-1146. doi: 10.1007/s11605-009-0855-1Dedemadi G, Georgoulis G, Kontopanos D, et al. Extragastroin-testinal stromal tumors of the omentum: review apropos of a case with a novel gain-of-function KIT mutation. J Gastrointest Cancer. 2009;40:73-78. doi: 10.1007/s12029-009-9089-4Giovanni C, Riccardo V, Grazia EM, et al. Laparoscopic treatment of an omental cyst: a case report and review of the literature. Surg Laparosc Endosc Percutan Tech. 2005;15(1):33-35.Katagiri H, Honjo K, Nasu M, et al. Omental infarction due to omental torsion. Case Rep Surg. 2013;2013:373810. doi: 10.1155/2013/373810Kranendonk MEG, van Herwaarden JA, Stupkova T, et al. Inflam-matory characteristics of distinct abdominal adipose tissue depots relate | Surgery_Schwartz. M, Petrakis G, et al. Rectus sheath hematoma in a single secondary care institution: a retrospective study. Hernia. 2015;19(3):509-512.Collins D, Hogan AM, O’Shea D, Winter DC. The omentum: ana-tomical, metabolic, and surgical aspects. J Gastrointest Surg. 2009;13:1138-1146. doi: 10.1007/s11605-009-0855-1Dedemadi G, Georgoulis G, Kontopanos D, et al. Extragastroin-testinal stromal tumors of the omentum: review apropos of a case with a novel gain-of-function KIT mutation. J Gastrointest Cancer. 2009;40:73-78. doi: 10.1007/s12029-009-9089-4Giovanni C, Riccardo V, Grazia EM, et al. Laparoscopic treatment of an omental cyst: a case report and review of the literature. Surg Laparosc Endosc Percutan Tech. 2005;15(1):33-35.Katagiri H, Honjo K, Nasu M, et al. Omental infarction due to omental torsion. Case Rep Surg. 2013;2013:373810. doi: 10.1155/2013/373810Kranendonk MEG, van Herwaarden JA, Stupkova T, et al. Inflam-matory characteristics of distinct abdominal adipose tissue depots relate |
Surgery_Schwartz_10312 | Surgery_Schwartz | Case Rep Surg. 2013;2013:373810. doi: 10.1155/2013/373810Kranendonk MEG, van Herwaarden JA, Stupkova T, et al. Inflam-matory characteristics of distinct abdominal adipose tissue depots relate differently to metabolic risk factors for cardiovascular disease. Atherosclerosis. 2017;239:419-427. doi: 10.1016/j.atherosclerosis.2015.01.035Morison R. Remarks on some functions of the Omentum. Br Med J. 1906;1(2350):76-78.Motie MR, Asadi M. Large omental cyst: a case report and review of the literature. Acta Medica Iranica. 2011;49(10):690-693.Mouaqit O, Jahid A, Ifrine L, et al. Primary omental gastrointestinal stromal tumors. Clin Res Hepatol Gastroenterol. 2011;35: 590-593. doi: http://dx.doi.org/10.1016/j.clinre.2010.11.012Occhionorelli S, Zese M, Cappellari L, et al. Acute abdomen due to primary omental torsion and infarction. Case Rep Surg. 2014;2014:208382. doi: 10.1155/2014/208382Park TU, Oh JH, Chang IT, et al. Omental infarction: case series and review of the literature. J Emerg | Surgery_Schwartz. Case Rep Surg. 2013;2013:373810. doi: 10.1155/2013/373810Kranendonk MEG, van Herwaarden JA, Stupkova T, et al. Inflam-matory characteristics of distinct abdominal adipose tissue depots relate differently to metabolic risk factors for cardiovascular disease. Atherosclerosis. 2017;239:419-427. doi: 10.1016/j.atherosclerosis.2015.01.035Morison R. Remarks on some functions of the Omentum. Br Med J. 1906;1(2350):76-78.Motie MR, Asadi M. Large omental cyst: a case report and review of the literature. Acta Medica Iranica. 2011;49(10):690-693.Mouaqit O, Jahid A, Ifrine L, et al. Primary omental gastrointestinal stromal tumors. Clin Res Hepatol Gastroenterol. 2011;35: 590-593. doi: http://dx.doi.org/10.1016/j.clinre.2010.11.012Occhionorelli S, Zese M, Cappellari L, et al. Acute abdomen due to primary omental torsion and infarction. Case Rep Surg. 2014;2014:208382. doi: 10.1155/2014/208382Park TU, Oh JH, Chang IT, et al. Omental infarction: case series and review of the literature. J Emerg |
Surgery_Schwartz_10313 | Surgery_Schwartz | primary omental torsion and infarction. Case Rep Surg. 2014;2014:208382. doi: 10.1155/2014/208382Park TU, Oh JH, Chang IT, et al. Omental infarction: case series and review of the literature. J Emerg Med. 2017;42:149-154. doi: 10.1016/j.jemermed.2008.07.023Platell C, Cooper D, Papadimitriou JM, Hall JC. The omentum. World J Gastroenterol. 2000;6:169-176. doi: 10.3748/wjg.v6.i2.169Rangel-Moreno J, Moyron-Quiroz JE, Carragher DM, et al. Omental milky spots develop in the absence of lymphoid tissue-inducer cells and support B and T cell responses to peritoneal antigens. Immunity. 2017;30:731-743. doi: 10.1016/j.immuni.2009.03.014Rao TN, Parvathi T, Suvarchala A. Omental lymphangioma in adults—rare presentation report of a case. Case Rep Surg. 2012;2012:629482. doi: 10.1155/2012/629482Schwartz R, Reames M, McGrath P, et al. Primary solid neoplasms of the greater omentum. Surgery. 1991;109:543-539.Seow-En I, Seow-Choen F, Lim TKH, Leow WQ. Primary omen-tal gastrointestinal stromal tumour | Surgery_Schwartz. primary omental torsion and infarction. Case Rep Surg. 2014;2014:208382. doi: 10.1155/2014/208382Park TU, Oh JH, Chang IT, et al. Omental infarction: case series and review of the literature. J Emerg Med. 2017;42:149-154. doi: 10.1016/j.jemermed.2008.07.023Platell C, Cooper D, Papadimitriou JM, Hall JC. The omentum. World J Gastroenterol. 2000;6:169-176. doi: 10.3748/wjg.v6.i2.169Rangel-Moreno J, Moyron-Quiroz JE, Carragher DM, et al. Omental milky spots develop in the absence of lymphoid tissue-inducer cells and support B and T cell responses to peritoneal antigens. Immunity. 2017;30:731-743. doi: 10.1016/j.immuni.2009.03.014Rao TN, Parvathi T, Suvarchala A. Omental lymphangioma in adults—rare presentation report of a case. Case Rep Surg. 2012;2012:629482. doi: 10.1155/2012/629482Schwartz R, Reames M, McGrath P, et al. Primary solid neoplasms of the greater omentum. Surgery. 1991;109:543-539.Seow-En I, Seow-Choen F, Lim TKH, Leow WQ. Primary omen-tal gastrointestinal stromal tumour |
Surgery_Schwartz_10314 | Surgery_Schwartz | R, Reames M, McGrath P, et al. Primary solid neoplasms of the greater omentum. Surgery. 1991;109:543-539.Seow-En I, Seow-Choen F, Lim TKH, Leow WQ. Primary omen-tal gastrointestinal stromal tumour (GIST) presenting with a large abdominal mass and spontaneous haemoperitoneum. BMJ Case Rep. 2014;2014:bcr2014205528. doi: 10.1136/bcr-2014-205528Tarantino G, Lobello R, Scopacasa F, et al. The contribution of omen-tal adipose tissue to adipokine concentrations in patients with the metabolic syndrome. Clin Invest Med. 2007;30:E192–E199.Uramatsu M, Saida Y, Nagao J, et al. Omental cyst: report of a case. Surg Today. 2001;31:1104-1106. doi: 10.1007/s595-001-8068-0Yoo E, Kim JH, Kim M-J, et al. Greater and lesser omenta: nor-mal anatomy and pathologic processes. RadioGraphics. 2007;27:707-720. doi: 10.1148/rg.273065085MesenteryAdams SD, Stanton MP. Malrotation and intestinal atre-sias. Early Hum Dev. 2017;90:921-925. doi: 10.1016/j.earlhumdev.2014.09.017Akram S, Pardi DS, Schaffner JA, Smyrk | Surgery_Schwartz. R, Reames M, McGrath P, et al. Primary solid neoplasms of the greater omentum. Surgery. 1991;109:543-539.Seow-En I, Seow-Choen F, Lim TKH, Leow WQ. Primary omen-tal gastrointestinal stromal tumour (GIST) presenting with a large abdominal mass and spontaneous haemoperitoneum. BMJ Case Rep. 2014;2014:bcr2014205528. doi: 10.1136/bcr-2014-205528Tarantino G, Lobello R, Scopacasa F, et al. The contribution of omen-tal adipose tissue to adipokine concentrations in patients with the metabolic syndrome. Clin Invest Med. 2007;30:E192–E199.Uramatsu M, Saida Y, Nagao J, et al. Omental cyst: report of a case. Surg Today. 2001;31:1104-1106. doi: 10.1007/s595-001-8068-0Yoo E, Kim JH, Kim M-J, et al. Greater and lesser omenta: nor-mal anatomy and pathologic processes. RadioGraphics. 2007;27:707-720. doi: 10.1148/rg.273065085MesenteryAdams SD, Stanton MP. Malrotation and intestinal atre-sias. Early Hum Dev. 2017;90:921-925. doi: 10.1016/j.earlhumdev.2014.09.017Akram S, Pardi DS, Schaffner JA, Smyrk |
Surgery_Schwartz_10315 | Surgery_Schwartz | 10.1148/rg.273065085MesenteryAdams SD, Stanton MP. Malrotation and intestinal atre-sias. Early Hum Dev. 2017;90:921-925. doi: 10.1016/j.earlhumdev.2014.09.017Akram S, Pardi DS, Schaffner JA, Smyrk TC. Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients. Clin Gastroenterol Hepatol. 2017;5: 589-596. doi: 10.1016/j.cgh.2007.02.032Avincsal MO, Otani K, Kanzawa M, et al. Sclerosing mes-enteritis: a real manifestation or histological mimic of Brunicardi_Ch35_p1549-p1566.indd 156412/02/19 9:59 AM 1565ABDOMINAL WALL, OMENTUM, MESENTERY, AND RETROPERITONEUMCHAPTER 35IgG4-related disease? Pathol Int. 2016;66(3):158-163. doi: 10.1111/pin.12386Coffey JC, O’Leary DP. The mesentery: structure, function, and role in disease. Lancet Gastroenterol Hepatol. 2017;1(3): 238-247. doi: 10.1016/S2468-1253(16)30026-7Daskalogiannaki M, Voloudaki A, Prassopoulos P, et al. CT evalu-ation of mesenteric panniculitis. Am J Roentgenol. 2000;174: 427-431. doi: | Surgery_Schwartz. 10.1148/rg.273065085MesenteryAdams SD, Stanton MP. Malrotation and intestinal atre-sias. Early Hum Dev. 2017;90:921-925. doi: 10.1016/j.earlhumdev.2014.09.017Akram S, Pardi DS, Schaffner JA, Smyrk TC. Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients. Clin Gastroenterol Hepatol. 2017;5: 589-596. doi: 10.1016/j.cgh.2007.02.032Avincsal MO, Otani K, Kanzawa M, et al. Sclerosing mes-enteritis: a real manifestation or histological mimic of Brunicardi_Ch35_p1549-p1566.indd 156412/02/19 9:59 AM 1565ABDOMINAL WALL, OMENTUM, MESENTERY, AND RETROPERITONEUMCHAPTER 35IgG4-related disease? Pathol Int. 2016;66(3):158-163. doi: 10.1111/pin.12386Coffey JC, O’Leary DP. The mesentery: structure, function, and role in disease. Lancet Gastroenterol Hepatol. 2017;1(3): 238-247. doi: 10.1016/S2468-1253(16)30026-7Daskalogiannaki M, Voloudaki A, Prassopoulos P, et al. CT evalu-ation of mesenteric panniculitis. Am J Roentgenol. 2000;174: 427-431. doi: |
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Surgery_Schwartz_10317 | Surgery_Schwartz | CY, et al. Colonic volvulus in the United States: trends, outcomes, and predictors of mortality. Ann Surg. 2014;259(2):293-301.Horton KM, Lawler LP, Fishman EK. CT findings in sclerosing mesenteritis (panniculitis): spectrum of disease. RadioGraph-ics. 2003;23:1561-1567. doi: 10.1148/rg.1103035010Hussein MRA, Abdelwahed SR. Mesenteric panniculitis: an update. Exp Rev Gastroenterol Hepatol. 2015;9:67-78. doi: 10.1586/17474124.2014.939632Kikiros CS, Edis AJ. Mesenteric panniculitis resulting in bowel obstruction: response to steroids. N Z J Surg. 1989;59(3): 287-290. doi: 10.1111/j.1445-2197.1989.tb01565.xKirby R, Rajasagaram N, Ghusn M. Primary mesenteric gastroin-testinal stromal tumour. J Surg Case Rep. 2014;2014:rju050. doi: 10.1093/jscr/rju050Park I-S, Kye B-H, Kim H-S, et al. Primary mesenteric carcinoid tumor. J Korean Surg Soc. 2013;84:114-117. doi: 10.4174/jkss.2013.84.2.114Parra-Davila E, McKenney M, Sleeman D, et al. Mesenteric panniculitis: case report and literature | Surgery_Schwartz. CY, et al. Colonic volvulus in the United States: trends, outcomes, and predictors of mortality. Ann Surg. 2014;259(2):293-301.Horton KM, Lawler LP, Fishman EK. CT findings in sclerosing mesenteritis (panniculitis): spectrum of disease. RadioGraph-ics. 2003;23:1561-1567. doi: 10.1148/rg.1103035010Hussein MRA, Abdelwahed SR. Mesenteric panniculitis: an update. Exp Rev Gastroenterol Hepatol. 2015;9:67-78. doi: 10.1586/17474124.2014.939632Kikiros CS, Edis AJ. Mesenteric panniculitis resulting in bowel obstruction: response to steroids. N Z J Surg. 1989;59(3): 287-290. doi: 10.1111/j.1445-2197.1989.tb01565.xKirby R, Rajasagaram N, Ghusn M. Primary mesenteric gastroin-testinal stromal tumour. J Surg Case Rep. 2014;2014:rju050. doi: 10.1093/jscr/rju050Park I-S, Kye B-H, Kim H-S, et al. Primary mesenteric carcinoid tumor. J Korean Surg Soc. 2013;84:114-117. doi: 10.4174/jkss.2013.84.2.114Parra-Davila E, McKenney M, Sleeman D, et al. Mesenteric panniculitis: case report and literature |
Surgery_Schwartz_10318 | Surgery_Schwartz | mesenteric carcinoid tumor. J Korean Surg Soc. 2013;84:114-117. doi: 10.4174/jkss.2013.84.2.114Parra-Davila E, McKenney M, Sleeman D, et al. Mesenteric panniculitis: case report and literature review. Am Surg. 1998;64:768-771.Peng L, Yang L, Wu N, Wu B. Primary primitive neuroectoder-mal tumor arising in the mesentery and ileocecum: a report of three cases and review of the literature. Exp Ther Med. 2015;9(4):1299-1303. doi: 10.3892/etm.2015.2242Rajendran S, Khan A, Murphy M, O’Hanlon D. The diagnosis and treatment of a symptomatic mesenteric cyst. BMJ Case Reports. 2014;2014:bcr2013202410. doi: 10.1136/bcr-2013-202410Salar O, El-Sharkawy AM, Singh R, Speake W. Internal her-nias: a brief review. Hernia. 2013;17:373-377. doi: 10.1007/s10029-012-1023-1Tan JJ-Y, Tan K-K, Chew S-P. Mesenteric cysts: an institution experience over 14 years and review of literature. World J Surg. 2009;33:1961-1965. doi: 10.1007/s00268-009-0133-0van Putte-Katier N, van Bommel EFH, Elgersma OE, Hendriksz TR. | Surgery_Schwartz. mesenteric carcinoid tumor. J Korean Surg Soc. 2013;84:114-117. doi: 10.4174/jkss.2013.84.2.114Parra-Davila E, McKenney M, Sleeman D, et al. Mesenteric panniculitis: case report and literature review. Am Surg. 1998;64:768-771.Peng L, Yang L, Wu N, Wu B. Primary primitive neuroectoder-mal tumor arising in the mesentery and ileocecum: a report of three cases and review of the literature. Exp Ther Med. 2015;9(4):1299-1303. doi: 10.3892/etm.2015.2242Rajendran S, Khan A, Murphy M, O’Hanlon D. The diagnosis and treatment of a symptomatic mesenteric cyst. BMJ Case Reports. 2014;2014:bcr2013202410. doi: 10.1136/bcr-2013-202410Salar O, El-Sharkawy AM, Singh R, Speake W. Internal her-nias: a brief review. Hernia. 2013;17:373-377. doi: 10.1007/s10029-012-1023-1Tan JJ-Y, Tan K-K, Chew S-P. Mesenteric cysts: an institution experience over 14 years and review of literature. World J Surg. 2009;33:1961-1965. doi: 10.1007/s00268-009-0133-0van Putte-Katier N, van Bommel EFH, Elgersma OE, Hendriksz TR. |
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Surgery_Schwartz_10320 | Surgery_Schwartz | a tertiary care center in Taiwan. Urol Sci. 2015;26:218-221. doi: http://dx.doi.org/10.1016/j.urols.2015.01.009Kermani TA, Crowson CS, Achenbach SJ, Luthra HS. Idio-pathic retroperitoneal fibrosis: a retrospective review of clini-cal presentation, treatment, and outcomes. Mayo Clin Proc. 2011;86(4):297-303. doi: 10.4065/mcp.2010.0663Knezevic S, Ignjatovic I, Lukic S, et al. Primary retroperitoneal mucinous cystadenoma: a case report. World J Gastroenterol. 2015;21(17):5427-5431. doi: 10.3748/wjg.v21.i17.5427Martorana D, Vaglio A, Greco P, et al. Chronic periaortitis and HLA–DRB1*03: another clue to an autoimmune origin. Arthri-tis Care Res. 2006;55:126-130. doi: 10.1002/art.21698Messiou C, Moskovic E, Vanel D, et al. Primary retroperitoneal soft tissue sarcoma: imaging appearances, pitfalls and diag-nostic algorithm. Eur J Surg Oncol. 2017;43(7):1191-1198. doi: 10.1016/j.ejso.2016.10.032Miralas P, Skandalakis J. Surgical Anatomy of the retroperitoneal spaces part II: the architecture | Surgery_Schwartz. a tertiary care center in Taiwan. Urol Sci. 2015;26:218-221. doi: http://dx.doi.org/10.1016/j.urols.2015.01.009Kermani TA, Crowson CS, Achenbach SJ, Luthra HS. Idio-pathic retroperitoneal fibrosis: a retrospective review of clini-cal presentation, treatment, and outcomes. Mayo Clin Proc. 2011;86(4):297-303. doi: 10.4065/mcp.2010.0663Knezevic S, Ignjatovic I, Lukic S, et al. Primary retroperitoneal mucinous cystadenoma: a case report. World J Gastroenterol. 2015;21(17):5427-5431. doi: 10.3748/wjg.v21.i17.5427Martorana D, Vaglio A, Greco P, et al. Chronic periaortitis and HLA–DRB1*03: another clue to an autoimmune origin. Arthri-tis Care Res. 2006;55:126-130. doi: 10.1002/art.21698Messiou C, Moskovic E, Vanel D, et al. Primary retroperitoneal soft tissue sarcoma: imaging appearances, pitfalls and diag-nostic algorithm. Eur J Surg Oncol. 2017;43(7):1191-1198. doi: 10.1016/j.ejso.2016.10.032Miralas P, Skandalakis J. Surgical Anatomy of the retroperitoneal spaces part II: the architecture |
Surgery_Schwartz_10321 | Surgery_Schwartz | and diag-nostic algorithm. Eur J Surg Oncol. 2017;43(7):1191-1198. doi: 10.1016/j.ejso.2016.10.032Miralas P, Skandalakis J. Surgical Anatomy of the retroperitoneal spaces part II: the architecture of the retroperitoneal space. Am Surg. 2010;76(1):33-42.Osman S, Lehnert B, Eloseimy S, et al. A comprehensive review of the retroperitoneal anatomy, neoplasms, and pattern of disease spread. Curr Probl Diagn Radiol. 2013;42(5):191-208.Scali EP, Chandler TM, Heffernan EJ, Coyle J, Harris AC, Chang SD. Primary retroperitoneal masses: what is the differential diagnosis? Abdom Imaging. 2015;40:1887-1903. doi: 10.1007/s00261-014-0311-xScheel P, Feeley N. Retroperitoneal fibrosis: the clinical, labora-tory, and radiographic presentation. Medicine (Baltimore). 2009;88(4):202-207.Stone JR. Aortitis, periaortitis, and retroperitoneal fibrosis, as mani-festations of IgG4-related systemic disease. Curr Opin Rheu-matol. 2011;23(1):88-94.Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. | Surgery_Schwartz. and diag-nostic algorithm. Eur J Surg Oncol. 2017;43(7):1191-1198. doi: 10.1016/j.ejso.2016.10.032Miralas P, Skandalakis J. Surgical Anatomy of the retroperitoneal spaces part II: the architecture of the retroperitoneal space. Am Surg. 2010;76(1):33-42.Osman S, Lehnert B, Eloseimy S, et al. A comprehensive review of the retroperitoneal anatomy, neoplasms, and pattern of disease spread. Curr Probl Diagn Radiol. 2013;42(5):191-208.Scali EP, Chandler TM, Heffernan EJ, Coyle J, Harris AC, Chang SD. Primary retroperitoneal masses: what is the differential diagnosis? Abdom Imaging. 2015;40:1887-1903. doi: 10.1007/s00261-014-0311-xScheel P, Feeley N. Retroperitoneal fibrosis: the clinical, labora-tory, and radiographic presentation. Medicine (Baltimore). 2009;88(4):202-207.Stone JR. Aortitis, periaortitis, and retroperitoneal fibrosis, as mani-festations of IgG4-related systemic disease. Curr Opin Rheu-matol. 2011;23(1):88-94.Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. |
Surgery_Schwartz_10322 | Surgery_Schwartz | periaortitis, and retroperitoneal fibrosis, as mani-festations of IgG4-related systemic disease. Curr Opin Rheu-matol. 2011;23(1):88-94.Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet. 2017;367(9506):241-251. doi: 10.1016/S0140-6736(06)68035-5van Bommel EFH, Siemes C, Hak LE, et al. Long-term renal and patient outcome in idiopathic retroperitoneal fibrosis treated with prednisone. Am J Kidney Dis. 2017;49:615-625. doi: 10.1053/j.ajkd.2007.02.268Brunicardi_Ch35_p1549-p1566.indd 156512/02/19 9:59 AM | Surgery_Schwartz. periaortitis, and retroperitoneal fibrosis, as mani-festations of IgG4-related systemic disease. Curr Opin Rheu-matol. 2011;23(1):88-94.Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet. 2017;367(9506):241-251. doi: 10.1016/S0140-6736(06)68035-5van Bommel EFH, Siemes C, Hak LE, et al. Long-term renal and patient outcome in idiopathic retroperitoneal fibrosis treated with prednisone. Am J Kidney Dis. 2017;49:615-625. doi: 10.1053/j.ajkd.2007.02.268Brunicardi_Ch35_p1549-p1566.indd 156512/02/19 9:59 AM |
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Surgery_Schwartz_10324 | Surgery_Schwartz | INTRODUCTIONSarcomas are a heterogeneous group of neoplasms that arise predominantly from cells of the embryonic mesoderm. While the majority of sarcomas are soft tissue sarcomas, other types of sarcoma include bone sarcomas (osteosarcoma, chondrosarcoma, and rare bone tumors like chordoma, angiosar-coma, and leiomyosarcoma of bone) and Ewing’s sarcoma/peripheral primitive neuroectodermal tumor, which can occur either in the bone or in the soft tissues. The primary focus of this chapter is soft tissue sarcomas. Most primary soft tissue sarco-mas originate in an extremity (50–60%); the next most common sites are the trunk (19%), retroperitoneum (15%), and head and neck (9%). The anatomic site of a primary sarcoma influ-ences treatment and outcome.1Soft tissue sarcomas include more than 70 histologic sub-types (Table 36-1). Historically, the most common subtypes in adults (excluding Kaposi’s sarcoma) were malignant fibrous histiocytoma (28%), liposarcoma (15%), leiomyosarcoma (12%), | Surgery_Schwartz. INTRODUCTIONSarcomas are a heterogeneous group of neoplasms that arise predominantly from cells of the embryonic mesoderm. While the majority of sarcomas are soft tissue sarcomas, other types of sarcoma include bone sarcomas (osteosarcoma, chondrosarcoma, and rare bone tumors like chordoma, angiosar-coma, and leiomyosarcoma of bone) and Ewing’s sarcoma/peripheral primitive neuroectodermal tumor, which can occur either in the bone or in the soft tissues. The primary focus of this chapter is soft tissue sarcomas. Most primary soft tissue sarco-mas originate in an extremity (50–60%); the next most common sites are the trunk (19%), retroperitoneum (15%), and head and neck (9%). The anatomic site of a primary sarcoma influ-ences treatment and outcome.1Soft tissue sarcomas include more than 70 histologic sub-types (Table 36-1). Historically, the most common subtypes in adults (excluding Kaposi’s sarcoma) were malignant fibrous histiocytoma (28%), liposarcoma (15%), leiomyosarcoma (12%), |
Surgery_Schwartz_10325 | Surgery_Schwartz | 70 histologic sub-types (Table 36-1). Historically, the most common subtypes in adults (excluding Kaposi’s sarcoma) were malignant fibrous histiocytoma (28%), liposarcoma (15%), leiomyosarcoma (12%), synovial sarcoma (10%), and malignant peripheral nerve sheath tumor (6%).2 Today, malignant fibrous histiocytoma is classified as either leiomyosarcoma, pleomorphic undifferenti-ated sarcoma, myxofibrosarcoma, or dedifferentiated liposar-coma based on cellular differentiation and genetics. Embryonal/alveolar rhabdomyosarcomas are the most common soft tissue sarcomas of childhood, whereas pleomorphic rhabdomyosar-coma occurs predominantly in adults, and although it shares part 12of the name, it has a different biology and should not be treated as a pediatric sarcoma.During the past 25 years, patients with extremity sarcomas have been treated with a multimodality approach, which has led to some improvements in survival, local control, and quality of life.3 However, patients with abdominal | Surgery_Schwartz. 70 histologic sub-types (Table 36-1). Historically, the most common subtypes in adults (excluding Kaposi’s sarcoma) were malignant fibrous histiocytoma (28%), liposarcoma (15%), leiomyosarcoma (12%), synovial sarcoma (10%), and malignant peripheral nerve sheath tumor (6%).2 Today, malignant fibrous histiocytoma is classified as either leiomyosarcoma, pleomorphic undifferenti-ated sarcoma, myxofibrosarcoma, or dedifferentiated liposar-coma based on cellular differentiation and genetics. Embryonal/alveolar rhabdomyosarcomas are the most common soft tissue sarcomas of childhood, whereas pleomorphic rhabdomyosar-coma occurs predominantly in adults, and although it shares part 12of the name, it has a different biology and should not be treated as a pediatric sarcoma.During the past 25 years, patients with extremity sarcomas have been treated with a multimodality approach, which has led to some improvements in survival, local control, and quality of life.3 However, patients with abdominal |
Surgery_Schwartz_10326 | Surgery_Schwartz | patients with extremity sarcomas have been treated with a multimodality approach, which has led to some improvements in survival, local control, and quality of life.3 However, patients with abdominal sarcomas con-tinue to have high rates of recurrence and poor overall survival.4 The overall 5-year survival rate for patients with all stages of soft tissue sarcoma is 50% to 60%. Of the patients who die of sarcoma, most succumb to lung metastasis, which 80% of the time occurs within 2 to 3 years after initial diagnosis.INCIDENCEIn the United States in 2012, approximately 11,280 new cases of soft tissue sarcoma were diagnosed, and 3900 deaths were attributable to this disease.5 The incidence of soft tissue sarcomas increased 1.3% per year from 1995 to 2009, and subsequently leveled off while mortality rates increased slightly (0.5% per year) from 2001 to 2014.5 The true incidence of sarcoma is thought to be higher than reported, and gastrointestinal stromal tumors (GISTs) likely account | Surgery_Schwartz. patients with extremity sarcomas have been treated with a multimodality approach, which has led to some improvements in survival, local control, and quality of life.3 However, patients with abdominal sarcomas con-tinue to have high rates of recurrence and poor overall survival.4 The overall 5-year survival rate for patients with all stages of soft tissue sarcoma is 50% to 60%. Of the patients who die of sarcoma, most succumb to lung metastasis, which 80% of the time occurs within 2 to 3 years after initial diagnosis.INCIDENCEIn the United States in 2012, approximately 11,280 new cases of soft tissue sarcoma were diagnosed, and 3900 deaths were attributable to this disease.5 The incidence of soft tissue sarcomas increased 1.3% per year from 1995 to 2009, and subsequently leveled off while mortality rates increased slightly (0.5% per year) from 2001 to 2014.5 The true incidence of sarcoma is thought to be higher than reported, and gastrointestinal stromal tumors (GISTs) likely account |
Surgery_Schwartz_10327 | Surgery_Schwartz | mortality rates increased slightly (0.5% per year) from 2001 to 2014.5 The true incidence of sarcoma is thought to be higher than reported, and gastrointestinal stromal tumors (GISTs) likely account for an additional 5000 new sarcoma cases per year.1 Overall, sarcomas affect 5 to 6 individuals per 100,000 inhabitants per year,6 accounting for less than 1% of all malignancies in adults and 15% of malignancies in children.7345Soft Tissue SarcomasRicardo J. Gonzalez, Alessandro Gronchi, and Raphael E. Pollock 36chapterIntroduction1567Incidence1567Epidemiology1568Radiation Exposure / 1568Occupational Chemical Exposure / 1568Trauma / 1568Chronic Lymphedema / 1568Molecular Pathogenesis1569Translocation-Associated Sarcomas / 1569Amplification-Associated Sarcomas / 1569Oncogenic Mutations / 1569Complex Genomic Rearrangements / 1570Initial Assessment1570Clinical Presentation / 1570Diagnostic Imaging / 1570Biopsy Techniques / 1571Pathologic Assessment and Classification / 1572Staging and | Surgery_Schwartz. mortality rates increased slightly (0.5% per year) from 2001 to 2014.5 The true incidence of sarcoma is thought to be higher than reported, and gastrointestinal stromal tumors (GISTs) likely account for an additional 5000 new sarcoma cases per year.1 Overall, sarcomas affect 5 to 6 individuals per 100,000 inhabitants per year,6 accounting for less than 1% of all malignancies in adults and 15% of malignancies in children.7345Soft Tissue SarcomasRicardo J. Gonzalez, Alessandro Gronchi, and Raphael E. Pollock 36chapterIntroduction1567Incidence1567Epidemiology1568Radiation Exposure / 1568Occupational Chemical Exposure / 1568Trauma / 1568Chronic Lymphedema / 1568Molecular Pathogenesis1569Translocation-Associated Sarcomas / 1569Amplification-Associated Sarcomas / 1569Oncogenic Mutations / 1569Complex Genomic Rearrangements / 1570Initial Assessment1570Clinical Presentation / 1570Diagnostic Imaging / 1570Biopsy Techniques / 1571Pathologic Assessment and Classification / 1572Staging and |
Surgery_Schwartz_10328 | Surgery_Schwartz | 1569Complex Genomic Rearrangements / 1570Initial Assessment1570Clinical Presentation / 1570Diagnostic Imaging / 1570Biopsy Techniques / 1571Pathologic Assessment and Classification / 1572Staging and Prognostic Factors / 1573Treatment of Extremity and Trunk Wall Sarcoma1574Surgery / 1575Radiation Therapy / 1577Systemic Therapy / 1578Concurrent Chemoradiation Therapy / 1580Posttreatment Surveillance / 1580Management of Recurrent Sarcoma / 1581Special Clinical Situations1582Myxoid Liposarcoma / 1582Retroperitoneal Sarcoma / 1582Gastrointestinal Sarcoma / 1583Breast Sarcoma / 1584Uterine Sarcoma / 1584Gastrointestinal Stromal Tumors1585Radiologic Assessment / 1585Management of Localized Disease / 1585Management of Locally Advanced or Metastatic Disease / 1585Multidisciplinary Treatment / 1586Postoperative Imatinib / 1586Preoperative Imatinib / 1587Desmoids1588Dermatofibrosarcoma Protuberans1588Pediatric Sarcomas1589Rhabdomyosarcoma / 1589Nonrhabdomyosarcoma Soft Tissue Sarcomas / | Surgery_Schwartz. 1569Complex Genomic Rearrangements / 1570Initial Assessment1570Clinical Presentation / 1570Diagnostic Imaging / 1570Biopsy Techniques / 1571Pathologic Assessment and Classification / 1572Staging and Prognostic Factors / 1573Treatment of Extremity and Trunk Wall Sarcoma1574Surgery / 1575Radiation Therapy / 1577Systemic Therapy / 1578Concurrent Chemoradiation Therapy / 1580Posttreatment Surveillance / 1580Management of Recurrent Sarcoma / 1581Special Clinical Situations1582Myxoid Liposarcoma / 1582Retroperitoneal Sarcoma / 1582Gastrointestinal Sarcoma / 1583Breast Sarcoma / 1584Uterine Sarcoma / 1584Gastrointestinal Stromal Tumors1585Radiologic Assessment / 1585Management of Localized Disease / 1585Management of Locally Advanced or Metastatic Disease / 1585Multidisciplinary Treatment / 1586Postoperative Imatinib / 1586Preoperative Imatinib / 1587Desmoids1588Dermatofibrosarcoma Protuberans1588Pediatric Sarcomas1589Rhabdomyosarcoma / 1589Nonrhabdomyosarcoma Soft Tissue Sarcomas / |
Surgery_Schwartz_10329 | Surgery_Schwartz | / 1586Postoperative Imatinib / 1586Preoperative Imatinib / 1587Desmoids1588Dermatofibrosarcoma Protuberans1588Pediatric Sarcomas1589Rhabdomyosarcoma / 1589Nonrhabdomyosarcoma Soft Tissue Sarcomas / 1589Research Perspectives1590Conclusions1590Brunicardi_Ch36_p1567-p1598.indd 156701/03/19 6:38 PM 1568Table 36-1Relative frequency of histologic subtypes of soft tissue sarcomaHISTOLOGIC SUBTYPESNO.%Liposarcoma18815Leiomyosarcoma14812Unclassified sarcoma14011Synovial sarcoma12510Malignant peripheral nerve sheath tumor726Rhabdomyosarcoma605Fibrosarcoma383Ewing sarcoma252Angiosarcoma252Osteosarcoma141Epithelioid sarcoma141Chondrosarcoma131Clear cell sarcoma121Alveolar soft part sarcoma71Malignant hemangiopericytoma50.4Data from Coindre JM, Terrier P, Guillou L, et al. Predictive value of grade for metastasis development in the main histologic types of adult soft tissue sarcomas: a study of 1240 patients from the French Federation of Cancer Centers Sarcoma Group, Cancer. 2001 May | Surgery_Schwartz. / 1586Postoperative Imatinib / 1586Preoperative Imatinib / 1587Desmoids1588Dermatofibrosarcoma Protuberans1588Pediatric Sarcomas1589Rhabdomyosarcoma / 1589Nonrhabdomyosarcoma Soft Tissue Sarcomas / 1589Research Perspectives1590Conclusions1590Brunicardi_Ch36_p1567-p1598.indd 156701/03/19 6:38 PM 1568Table 36-1Relative frequency of histologic subtypes of soft tissue sarcomaHISTOLOGIC SUBTYPESNO.%Liposarcoma18815Leiomyosarcoma14812Unclassified sarcoma14011Synovial sarcoma12510Malignant peripheral nerve sheath tumor726Rhabdomyosarcoma605Fibrosarcoma383Ewing sarcoma252Angiosarcoma252Osteosarcoma141Epithelioid sarcoma141Chondrosarcoma131Clear cell sarcoma121Alveolar soft part sarcoma71Malignant hemangiopericytoma50.4Data from Coindre JM, Terrier P, Guillou L, et al. Predictive value of grade for metastasis development in the main histologic types of adult soft tissue sarcomas: a study of 1240 patients from the French Federation of Cancer Centers Sarcoma Group, Cancer. 2001 May |
Surgery_Schwartz_10330 | Surgery_Schwartz | of grade for metastasis development in the main histologic types of adult soft tissue sarcomas: a study of 1240 patients from the French Federation of Cancer Centers Sarcoma Group, Cancer. 2001 May 15;91(10):1914-1926.EPIDEMIOLOGYExcept for malignant peripheral nerve sheath tumors in patients with neurofibromatosis, sarcomas do not seem to result from progression or dedifferentiation of a benign soft tissue tumor. While most sarcomas are of unknown cause, a few sarcoma sub-types have been observed in settings suggesting etiology.Radiation ExposureExternal radiation therapy is a rare but well-established risk factor for soft tissue sarcoma that may be associated with radiation-induced mutations of the p53 gene.8 The incidence of sarcoma among patients who are often treated with radiation for cancer of the breast, cervix, ovary, testes, or lymphatic system is 8 to 50 times the general-population risk.9,10 In a review of 160 patients with postirradiation sarcomas, the most common | Surgery_Schwartz. of grade for metastasis development in the main histologic types of adult soft tissue sarcomas: a study of 1240 patients from the French Federation of Cancer Centers Sarcoma Group, Cancer. 2001 May 15;91(10):1914-1926.EPIDEMIOLOGYExcept for malignant peripheral nerve sheath tumors in patients with neurofibromatosis, sarcomas do not seem to result from progression or dedifferentiation of a benign soft tissue tumor. While most sarcomas are of unknown cause, a few sarcoma sub-types have been observed in settings suggesting etiology.Radiation ExposureExternal radiation therapy is a rare but well-established risk factor for soft tissue sarcoma that may be associated with radiation-induced mutations of the p53 gene.8 The incidence of sarcoma among patients who are often treated with radiation for cancer of the breast, cervix, ovary, testes, or lymphatic system is 8 to 50 times the general-population risk.9,10 In a review of 160 patients with postirradiation sarcomas, the most common |
Surgery_Schwartz_10331 | Surgery_Schwartz | for cancer of the breast, cervix, ovary, testes, or lymphatic system is 8 to 50 times the general-population risk.9,10 In a review of 160 patients with postirradiation sarcomas, the most common histologic types were osteogenic sarcoma, pleomorphic undifferentiated sarcoma, angiosarcoma, and lymphangiosarcoma.9 The risk of developing a sarcoma increased with radiation dose, and the median time between radiation therapy and diagnosis of sarcoma was 10 years.9 A review of 44 patients with radiation-associated sarcomas identified between 1989 and 2009 noted that the average period from initial radiation treatment to diagnosis was 16 years and that radiation-associated sarcomas occurred most commonly in patients treated for breast cancer (36% of the patients in the series) and lymphoma (34% of the patients in the series).11 The 5-year overall survival rate for patients presenting without metastasis was 44%. A recent review of undifferentiated pleomorphic sarcoma characterizing outcomes | Surgery_Schwartz. for cancer of the breast, cervix, ovary, testes, or lymphatic system is 8 to 50 times the general-population risk.9,10 In a review of 160 patients with postirradiation sarcomas, the most common histologic types were osteogenic sarcoma, pleomorphic undifferentiated sarcoma, angiosarcoma, and lymphangiosarcoma.9 The risk of developing a sarcoma increased with radiation dose, and the median time between radiation therapy and diagnosis of sarcoma was 10 years.9 A review of 44 patients with radiation-associated sarcomas identified between 1989 and 2009 noted that the average period from initial radiation treatment to diagnosis was 16 years and that radiation-associated sarcomas occurred most commonly in patients treated for breast cancer (36% of the patients in the series) and lymphoma (34% of the patients in the series).11 The 5-year overall survival rate for patients presenting without metastasis was 44%. A recent review of undifferentiated pleomorphic sarcoma characterizing outcomes |
Surgery_Schwartz_10332 | Surgery_Schwartz | of the patients in the series).11 The 5-year overall survival rate for patients presenting without metastasis was 44%. A recent review of undifferentiated pleomorphic sarcoma characterizing outcomes between sporadic and radiation-associated presentation identified a higher incidence of local recurrence and worse overall and disease specific survival among patients who presented with radiation-associated undifferentiated pleomorphic sarcoma.12Occupational Chemical ExposureExposure to herbicides such as phenoxyacetic acids and to wood preservatives containing chlorophenols has been linked to an increased risk of soft tissue sarcoma.13 Several chemi-cal carcinogens, including thorium oxide (Thorotrast), vinyl chloride, and arsenic, have been associated with hepatic angiosarcomas.14TraumaAlthough patients with sarcoma often report a history of trauma, no causal relationship has been established. More often, a minor injury calls attention to a preexisting tumor.Chronic LymphedemaIn 1948, | Surgery_Schwartz. of the patients in the series).11 The 5-year overall survival rate for patients presenting without metastasis was 44%. A recent review of undifferentiated pleomorphic sarcoma characterizing outcomes between sporadic and radiation-associated presentation identified a higher incidence of local recurrence and worse overall and disease specific survival among patients who presented with radiation-associated undifferentiated pleomorphic sarcoma.12Occupational Chemical ExposureExposure to herbicides such as phenoxyacetic acids and to wood preservatives containing chlorophenols has been linked to an increased risk of soft tissue sarcoma.13 Several chemi-cal carcinogens, including thorium oxide (Thorotrast), vinyl chloride, and arsenic, have been associated with hepatic angiosarcomas.14TraumaAlthough patients with sarcoma often report a history of trauma, no causal relationship has been established. More often, a minor injury calls attention to a preexisting tumor.Chronic LymphedemaIn 1948, |
Surgery_Schwartz_10333 | Surgery_Schwartz | patients with sarcoma often report a history of trauma, no causal relationship has been established. More often, a minor injury calls attention to a preexisting tumor.Chronic LymphedemaIn 1948, Stewart and Treves first described the association between chronic lymphedema after axillary dissection and sub-sequent lymphangiosarcoma (Fig. 36-1).15 Lymphangiosarcoma has been estimated to occur in 0.07% of patients who undergo axillary node dissection.16 It also has been reported to occur after filarial infections and in the lower extremities of patients with congenital lymphedema.17,18 Lymphangiosarcoma is gen-erally an aggressive tumor; average survival of patients with lymphangiosarcoma is 19 months.19Key Points1 Sarcomas are a heterogeneous group of tumors that can occur throughout the body and encompass more than 50 subtypes with distinct histologic lines of differentiation.2 Approximately two-thirds of soft tissue sarcomas arise in the extremities; the remaining one-third is | Surgery_Schwartz. patients with sarcoma often report a history of trauma, no causal relationship has been established. More often, a minor injury calls attention to a preexisting tumor.Chronic LymphedemaIn 1948, Stewart and Treves first described the association between chronic lymphedema after axillary dissection and sub-sequent lymphangiosarcoma (Fig. 36-1).15 Lymphangiosarcoma has been estimated to occur in 0.07% of patients who undergo axillary node dissection.16 It also has been reported to occur after filarial infections and in the lower extremities of patients with congenital lymphedema.17,18 Lymphangiosarcoma is gen-erally an aggressive tumor; average survival of patients with lymphangiosarcoma is 19 months.19Key Points1 Sarcomas are a heterogeneous group of tumors that can occur throughout the body and encompass more than 50 subtypes with distinct histologic lines of differentiation.2 Approximately two-thirds of soft tissue sarcomas arise in the extremities; the remaining one-third is |
Surgery_Schwartz_10334 | Surgery_Schwartz | the body and encompass more than 50 subtypes with distinct histologic lines of differentiation.2 Approximately two-thirds of soft tissue sarcomas arise in the extremities; the remaining one-third is distributed between the retroperitoneum, trunk, abdomen, head, and neck.3 Multimodality treatment, including surgical resection, radia-tion therapy, and, in selected cases, systemic chemotherapy, has been applied to patients with locally advanced, high-grade, extremity sarcomas.4 Overall 5-year survival rate for patients with all stages of soft tissue sarcoma is 50% to 60%.5 These rare tumors account for less than 1% of cancer in adults (estimated 10,000 cases per year in the United States) and represent 15% of cancers in children.6 The treatment algorithm for soft tissue sarcomas depends on tumor stage, site, and histology.7 Of the patients who die of sarcoma, most will succumb to metastatic disease in the lungs, which 80% of the time occurs within 2 to 3 years of the initial | Surgery_Schwartz. the body and encompass more than 50 subtypes with distinct histologic lines of differentiation.2 Approximately two-thirds of soft tissue sarcomas arise in the extremities; the remaining one-third is distributed between the retroperitoneum, trunk, abdomen, head, and neck.3 Multimodality treatment, including surgical resection, radia-tion therapy, and, in selected cases, systemic chemotherapy, has been applied to patients with locally advanced, high-grade, extremity sarcomas.4 Overall 5-year survival rate for patients with all stages of soft tissue sarcoma is 50% to 60%.5 These rare tumors account for less than 1% of cancer in adults (estimated 10,000 cases per year in the United States) and represent 15% of cancers in children.6 The treatment algorithm for soft tissue sarcomas depends on tumor stage, site, and histology.7 Of the patients who die of sarcoma, most will succumb to metastatic disease in the lungs, which 80% of the time occurs within 2 to 3 years of the initial |
Surgery_Schwartz_10335 | Surgery_Schwartz | depends on tumor stage, site, and histology.7 Of the patients who die of sarcoma, most will succumb to metastatic disease in the lungs, which 80% of the time occurs within 2 to 3 years of the initial diagnosis.8 Progress in the understanding of soft tissue sarcoma biology is crucial for the development of new treatments.Brunicardi_Ch36_p1567-p1598.indd 156801/03/19 6:38 PM 1569SOFT TISSUE SARCOMASCHAPTER 36Figure 36-1. A 57-year-old with a chronic, progressive lymph-edema of the left upper extremity developed lymphangiosarcoma 10 years after breast cancer treatment.Table 36-2Fusion transcripts in soft tissue sarcomaDIAGNOSISCHROMOSOMAL ABNORMALITYGENES INVOLVEDAlveolar rhabdomyosarcomat(2;13)(q35;q14)t(1;13)(p36;q14)PAX3-FKHRPAX7-FKHRAlveolar soft part sarcomat(X;17)(p11.2;q25)TFE3-ASPLAngiomatoid fibrous histiocytomat(12;16)(q13;p11)FUS-ATF1Clear cell sarcomat(12;22)(q13;q12)EWS-ATF1Congenital fibrosarcoma/congenital mesoblastic | Surgery_Schwartz. depends on tumor stage, site, and histology.7 Of the patients who die of sarcoma, most will succumb to metastatic disease in the lungs, which 80% of the time occurs within 2 to 3 years of the initial diagnosis.8 Progress in the understanding of soft tissue sarcoma biology is crucial for the development of new treatments.Brunicardi_Ch36_p1567-p1598.indd 156801/03/19 6:38 PM 1569SOFT TISSUE SARCOMASCHAPTER 36Figure 36-1. A 57-year-old with a chronic, progressive lymph-edema of the left upper extremity developed lymphangiosarcoma 10 years after breast cancer treatment.Table 36-2Fusion transcripts in soft tissue sarcomaDIAGNOSISCHROMOSOMAL ABNORMALITYGENES INVOLVEDAlveolar rhabdomyosarcomat(2;13)(q35;q14)t(1;13)(p36;q14)PAX3-FKHRPAX7-FKHRAlveolar soft part sarcomat(X;17)(p11.2;q25)TFE3-ASPLAngiomatoid fibrous histiocytomat(12;16)(q13;p11)FUS-ATF1Clear cell sarcomat(12;22)(q13;q12)EWS-ATF1Congenital fibrosarcoma/congenital mesoblastic |
Surgery_Schwartz_10336 | Surgery_Schwartz | soft part sarcomat(X;17)(p11.2;q25)TFE3-ASPLAngiomatoid fibrous histiocytomat(12;16)(q13;p11)FUS-ATF1Clear cell sarcomat(12;22)(q13;q12)EWS-ATF1Congenital fibrosarcoma/congenital mesoblastic nephromat(12;15)(p13;q25)ETV6-NTRK3Dermatofibrosarcoma protuberanst(17;22)(q22;q13)PDFGB-COL1A1Desmoplastic small round cell tumort(11;22)(p13;q12)EWS-WT1Endometrial stromal sarcomat(7;17)(p15;q21)JAZF1-JJAZ1Ewing’s sarcoma/peripheral primitive neuroectodermal tumort(11;22)(q24;q12)t(21;22)(q22;q12)t(7;22)(p22;q12)t(17;22)(q12;q12)t(2;22)(q33;q12)t(16;21)(p11;q22)EWS-FLI1EWS-ERGEWS-ETV1EWS-FEVEWS-E1AFFUS-ERGLow-grade fibromyxoid sarcomat(7;16)(q33;p11)FUS-CREB3I2Inflammatory myofibroblastic tumort(1;2)(q22;p23)t(2;19)(p23;p13)t(2;17)(p23;q23)TPM3-ALKTPM4-ALKCLTC-ALKMyxoid liposarcomat(12;16)(q13;p11)t(12;22)(q13;q12)TLS-CHOPEWS-CHOPMyxoid chondrosarcomat(9;22)(q22;q12)t(9;15)(q22;q21)t(9;17)(q22;q11)EWS-CHNTFC12-CHNTAF2N-CHNSynovial sarcomat(x;18)(p11;q11)SSX1-SYTSSX2-SYTSSX4-SYTMOLECULAR | Surgery_Schwartz. soft part sarcomat(X;17)(p11.2;q25)TFE3-ASPLAngiomatoid fibrous histiocytomat(12;16)(q13;p11)FUS-ATF1Clear cell sarcomat(12;22)(q13;q12)EWS-ATF1Congenital fibrosarcoma/congenital mesoblastic nephromat(12;15)(p13;q25)ETV6-NTRK3Dermatofibrosarcoma protuberanst(17;22)(q22;q13)PDFGB-COL1A1Desmoplastic small round cell tumort(11;22)(p13;q12)EWS-WT1Endometrial stromal sarcomat(7;17)(p15;q21)JAZF1-JJAZ1Ewing’s sarcoma/peripheral primitive neuroectodermal tumort(11;22)(q24;q12)t(21;22)(q22;q12)t(7;22)(p22;q12)t(17;22)(q12;q12)t(2;22)(q33;q12)t(16;21)(p11;q22)EWS-FLI1EWS-ERGEWS-ETV1EWS-FEVEWS-E1AFFUS-ERGLow-grade fibromyxoid sarcomat(7;16)(q33;p11)FUS-CREB3I2Inflammatory myofibroblastic tumort(1;2)(q22;p23)t(2;19)(p23;p13)t(2;17)(p23;q23)TPM3-ALKTPM4-ALKCLTC-ALKMyxoid liposarcomat(12;16)(q13;p11)t(12;22)(q13;q12)TLS-CHOPEWS-CHOPMyxoid chondrosarcomat(9;22)(q22;q12)t(9;15)(q22;q21)t(9;17)(q22;q11)EWS-CHNTFC12-CHNTAF2N-CHNSynovial sarcomat(x;18)(p11;q11)SSX1-SYTSSX2-SYTSSX4-SYTMOLECULAR |
Surgery_Schwartz_10337 | Surgery_Schwartz | chondrosarcomat(9;22)(q22;q12)t(9;15)(q22;q21)t(9;17)(q22;q11)EWS-CHNTFC12-CHNTAF2N-CHNSynovial sarcomat(x;18)(p11;q11)SSX1-SYTSSX2-SYTSSX4-SYTMOLECULAR PATHOGENESISSarcomas can be broadly classified into three groups accord-ing to the genetic events underlying their development: specific translocations or gene amplification, defining oncogenic muta-tions, and complex genomic rearrangements.20 In general, sar-comas resulting from identifiable molecular events tend to occur in younger patients with histology suggesting a clear line of differentiation. The identifiable molecular events include point mutations, translocations causing overexpression of an autocrine grow factor, and oncogenic fusion transcription factor produc-ing a cellular environment prone to malignant transformation. In contrast, sarcomas without identifiable genetic changes or expression profile signatures tend to occur in older patients and exhibit pleomorphic cytology and p53 dysfunction.21 Improved understanding of | Surgery_Schwartz. chondrosarcomat(9;22)(q22;q12)t(9;15)(q22;q21)t(9;17)(q22;q11)EWS-CHNTFC12-CHNTAF2N-CHNSynovial sarcomat(x;18)(p11;q11)SSX1-SYTSSX2-SYTSSX4-SYTMOLECULAR PATHOGENESISSarcomas can be broadly classified into three groups accord-ing to the genetic events underlying their development: specific translocations or gene amplification, defining oncogenic muta-tions, and complex genomic rearrangements.20 In general, sar-comas resulting from identifiable molecular events tend to occur in younger patients with histology suggesting a clear line of differentiation. The identifiable molecular events include point mutations, translocations causing overexpression of an autocrine grow factor, and oncogenic fusion transcription factor produc-ing a cellular environment prone to malignant transformation. In contrast, sarcomas without identifiable genetic changes or expression profile signatures tend to occur in older patients and exhibit pleomorphic cytology and p53 dysfunction.21 Improved understanding of |
Surgery_Schwartz_10338 | Surgery_Schwartz | sarcomas without identifiable genetic changes or expression profile signatures tend to occur in older patients and exhibit pleomorphic cytology and p53 dysfunction.21 Improved understanding of the molecular pathogenesis of sarcomas has revealed several potential targets against which investigators are working to develop subtype-specific targeted therapy.Translocation-Associated SarcomasTo date, translocations have been identified in 14 subtypes of soft tissue sarcoma, accounting for 20% to 30% of all sarcomas22 (Table 36-2). Translocations result in in-frame gene fusion, which in turn results in fused products encoding oncoproteins that function as transcriptional activators or repressors.23,24 The best characterized gene fusions are in Ewing’s sarcoma (EWS-FLI1), clear cell sarcoma (EWS-ATF1), myxoid/round cell liposarcoma (TLS-CHOP), alveolar rhabdomyosarcoma (PAX3-FHKR), desmoplastic small round cell tumor (EWS-WT1), and synovial sarcoma (SS18-SSX). Fusion gene–related sarcomas | Surgery_Schwartz. sarcomas without identifiable genetic changes or expression profile signatures tend to occur in older patients and exhibit pleomorphic cytology and p53 dysfunction.21 Improved understanding of the molecular pathogenesis of sarcomas has revealed several potential targets against which investigators are working to develop subtype-specific targeted therapy.Translocation-Associated SarcomasTo date, translocations have been identified in 14 subtypes of soft tissue sarcoma, accounting for 20% to 30% of all sarcomas22 (Table 36-2). Translocations result in in-frame gene fusion, which in turn results in fused products encoding oncoproteins that function as transcriptional activators or repressors.23,24 The best characterized gene fusions are in Ewing’s sarcoma (EWS-FLI1), clear cell sarcoma (EWS-ATF1), myxoid/round cell liposarcoma (TLS-CHOP), alveolar rhabdomyosarcoma (PAX3-FHKR), desmoplastic small round cell tumor (EWS-WT1), and synovial sarcoma (SS18-SSX). Fusion gene–related sarcomas |
Surgery_Schwartz_10339 | Surgery_Schwartz | myxoid/round cell liposarcoma (TLS-CHOP), alveolar rhabdomyosarcoma (PAX3-FHKR), desmoplastic small round cell tumor (EWS-WT1), and synovial sarcoma (SS18-SSX). Fusion gene–related sarcomas have been estimated to account for 30% or more of all sarcomas.25Direct or indirect interactions between fusion transcripts and cell cycle regulators have been elucidated by several inves-tigators and identify these transcripts as potentially promising molecular therapeutic targets.26 However, fusion genes in sar-coma have been successfully targeted in only a few cases, in which fusion resulted in overexpression of a growth factor or growth factor receptor. Several growth factors and their recep-tors (e.g., epidermal growth factor receptor) previously reported to play an important role in autocrine or paracrine stimulation of carcinoma growth have been associated with high histologic grade and poor prognosis in soft tissue sarcomas.Amplification-Associated SarcomasOncogenes are genes that can | Surgery_Schwartz. myxoid/round cell liposarcoma (TLS-CHOP), alveolar rhabdomyosarcoma (PAX3-FHKR), desmoplastic small round cell tumor (EWS-WT1), and synovial sarcoma (SS18-SSX). Fusion gene–related sarcomas have been estimated to account for 30% or more of all sarcomas.25Direct or indirect interactions between fusion transcripts and cell cycle regulators have been elucidated by several inves-tigators and identify these transcripts as potentially promising molecular therapeutic targets.26 However, fusion genes in sar-coma have been successfully targeted in only a few cases, in which fusion resulted in overexpression of a growth factor or growth factor receptor. Several growth factors and their recep-tors (e.g., epidermal growth factor receptor) previously reported to play an important role in autocrine or paracrine stimulation of carcinoma growth have been associated with high histologic grade and poor prognosis in soft tissue sarcomas.Amplification-Associated SarcomasOncogenes are genes that can |
Surgery_Schwartz_10340 | Surgery_Schwartz | or paracrine stimulation of carcinoma growth have been associated with high histologic grade and poor prognosis in soft tissue sarcomas.Amplification-Associated SarcomasOncogenes are genes that can induce malignant transformation and tend to drive cell proliferation. Several oncogenes have been associated with soft tissue sarcomas, including MDM2, N-myc, c-erbB2, and members of the ras family. These onco-genes produce specific oncoproteins that either play a role in nuclear function and cellular signal transduction or function as growth factors or growth factor receptors. This typically occurs in dedifferentiated liposarcoma, where the amplification of MDM2 drives the neoplastic process. Amplification of these genes has been shown to correlate with adverse outcome in sev-eral types of soft tissue sarcoma.23Oncogenic MutationsGISTs are the classic example of sarcomas in which tumorigen-esis is primarily driven by a single activating mutation, in the Brunicardi_Ch36_p1567-p1598.indd | Surgery_Schwartz. or paracrine stimulation of carcinoma growth have been associated with high histologic grade and poor prognosis in soft tissue sarcomas.Amplification-Associated SarcomasOncogenes are genes that can induce malignant transformation and tend to drive cell proliferation. Several oncogenes have been associated with soft tissue sarcomas, including MDM2, N-myc, c-erbB2, and members of the ras family. These onco-genes produce specific oncoproteins that either play a role in nuclear function and cellular signal transduction or function as growth factors or growth factor receptors. This typically occurs in dedifferentiated liposarcoma, where the amplification of MDM2 drives the neoplastic process. Amplification of these genes has been shown to correlate with adverse outcome in sev-eral types of soft tissue sarcoma.23Oncogenic MutationsGISTs are the classic example of sarcomas in which tumorigen-esis is primarily driven by a single activating mutation, in the Brunicardi_Ch36_p1567-p1598.indd |
Surgery_Schwartz_10341 | Surgery_Schwartz | tissue sarcoma.23Oncogenic MutationsGISTs are the classic example of sarcomas in which tumorigen-esis is primarily driven by a single activating mutation, in the Brunicardi_Ch36_p1567-p1598.indd 156901/03/19 6:38 PM 1570SPECIFIC CONSIDERATIONSPART IIgene encoding KIT receptor tyrosine kinase or platelet-derived growth factor receptor-α (PDGFRA).20 The majority of GISTs have mutations in either exon 11 or exon 9 of KIT and respond dramatically to the tyrosine kinase inhibitor imatinib mesylate, although this treatment rarely produces cure.Complex Genomic RearrangementsThe largest group of sarcomas is the group with complex cytoge-netic alterations, which includes high-grade spindle cell sarco-mas and pleomorphic sarcomas.20 Many sarcomas in this group exhibit inactivation of tumor suppressor genes. The two genes most relevant to soft tissue sarcoma are retinoblastoma (Rb) and p53. Mutations or deletions in Rb can lead to retinoblastoma, the most common malignant ocular neoplasm of | Surgery_Schwartz. tissue sarcoma.23Oncogenic MutationsGISTs are the classic example of sarcomas in which tumorigen-esis is primarily driven by a single activating mutation, in the Brunicardi_Ch36_p1567-p1598.indd 156901/03/19 6:38 PM 1570SPECIFIC CONSIDERATIONSPART IIgene encoding KIT receptor tyrosine kinase or platelet-derived growth factor receptor-α (PDGFRA).20 The majority of GISTs have mutations in either exon 11 or exon 9 of KIT and respond dramatically to the tyrosine kinase inhibitor imatinib mesylate, although this treatment rarely produces cure.Complex Genomic RearrangementsThe largest group of sarcomas is the group with complex cytoge-netic alterations, which includes high-grade spindle cell sarco-mas and pleomorphic sarcomas.20 Many sarcomas in this group exhibit inactivation of tumor suppressor genes. The two genes most relevant to soft tissue sarcoma are retinoblastoma (Rb) and p53. Mutations or deletions in Rb can lead to retinoblastoma, the most common malignant ocular neoplasm of |
Surgery_Schwartz_10342 | Surgery_Schwartz | genes. The two genes most relevant to soft tissue sarcoma are retinoblastoma (Rb) and p53. Mutations or deletions in Rb can lead to retinoblastoma, the most common malignant ocular neoplasm of childhood. Survi-vors of retinoblastoma are at risk for developing soft tissue and bone sarcomas later in life. Patients with germline mutations in p53 (Li-Fraumeni syndrome) have a high incidence of soft tissue sarcomas Mutant p53 expression is thought to correlate with poor overall survival.23 Strategies to target p53 mutation are being investigated for treatment of some sarcomas.Neurofibromatosis type 1 (von Recklinghausen’s disease) occurs in approximately 1 of every 3000 people and is due to various mutations in the NF-1 tumor suppressor gene, located on chromosome 17. Patients with neurofibromatosis type 1 have an estimated 3% to 15% additional risk of malignant disease compared with the general population lifetime risk, including malignant peripheral nerve sheath tumors (MPNST) and GIST. | Surgery_Schwartz. genes. The two genes most relevant to soft tissue sarcoma are retinoblastoma (Rb) and p53. Mutations or deletions in Rb can lead to retinoblastoma, the most common malignant ocular neoplasm of childhood. Survi-vors of retinoblastoma are at risk for developing soft tissue and bone sarcomas later in life. Patients with germline mutations in p53 (Li-Fraumeni syndrome) have a high incidence of soft tissue sarcomas Mutant p53 expression is thought to correlate with poor overall survival.23 Strategies to target p53 mutation are being investigated for treatment of some sarcomas.Neurofibromatosis type 1 (von Recklinghausen’s disease) occurs in approximately 1 of every 3000 people and is due to various mutations in the NF-1 tumor suppressor gene, located on chromosome 17. Patients with neurofibromatosis type 1 have an estimated 3% to 15% additional risk of malignant disease compared with the general population lifetime risk, including malignant peripheral nerve sheath tumors (MPNST) and GIST. |
Surgery_Schwartz_10343 | Surgery_Schwartz | type 1 have an estimated 3% to 15% additional risk of malignant disease compared with the general population lifetime risk, including malignant peripheral nerve sheath tumors (MPNST) and GIST. In turn, 25% to 50% of patients with MPNST have a mutation in NF-1.27INITIAL ASSESSMENTThe clinical behavior of most soft tissue sarcomas is determined by anatomic location (depth in relation to the investing fascia), histologic subtype and grade of aggressiveness, and size. The dominant pattern of metastasis is hematogenous, primarily to the lungs. Lymph node metastasis is rare (affecting <5% of patients) except in a few histologic subtypes, including epi-thelioid sarcoma, pediatric rhabdomyosarcoma, clear cell sar-coma, angiosarcoma, and, more rarely, synovial sarcoma and myxofibrosarcoma.28Clinical PresentationSoft tissue sarcomas most commonly present as an asymptom-atic mass. Extremity sarcomas may present as a deep venous thrombosis, particularly in patients without significant risk | Surgery_Schwartz. type 1 have an estimated 3% to 15% additional risk of malignant disease compared with the general population lifetime risk, including malignant peripheral nerve sheath tumors (MPNST) and GIST. In turn, 25% to 50% of patients with MPNST have a mutation in NF-1.27INITIAL ASSESSMENTThe clinical behavior of most soft tissue sarcomas is determined by anatomic location (depth in relation to the investing fascia), histologic subtype and grade of aggressiveness, and size. The dominant pattern of metastasis is hematogenous, primarily to the lungs. Lymph node metastasis is rare (affecting <5% of patients) except in a few histologic subtypes, including epi-thelioid sarcoma, pediatric rhabdomyosarcoma, clear cell sar-coma, angiosarcoma, and, more rarely, synovial sarcoma and myxofibrosarcoma.28Clinical PresentationSoft tissue sarcomas most commonly present as an asymptom-atic mass. Extremity sarcomas may present as a deep venous thrombosis, particularly in patients without significant risk |
Surgery_Schwartz_10344 | Surgery_Schwartz | PresentationSoft tissue sarcomas most commonly present as an asymptom-atic mass. Extremity sarcomas may present as a deep venous thrombosis, particularly in patients without significant risk factors for thrombosis.29 Tumors in the distal extremities are generally smaller, whereas tumors in the proximal extremities and retroperitoneum can grow quite large before becoming apparent. Tumors often grow centrifugally and can compress surrounding normal structures. Infrequently, tumor impinge-ment on bone or neurovascular bundles produces pain, edema, and swelling. Less frequently, tumors cause obstructive gastro-intestinal symptoms or neurologic symptoms related to com-pression of lumbar or pelvic nerves. Often an extremity mass is discovered after a traumatic event that draws attention to a preexisting lesion.The differential diagnosis of a soft tissue mass should include consideration of lipoma (which is 100 times more com-mon than sarcoma), lymphangioma, leiomyoma, neurinoma, primary or | Surgery_Schwartz. PresentationSoft tissue sarcomas most commonly present as an asymptom-atic mass. Extremity sarcomas may present as a deep venous thrombosis, particularly in patients without significant risk factors for thrombosis.29 Tumors in the distal extremities are generally smaller, whereas tumors in the proximal extremities and retroperitoneum can grow quite large before becoming apparent. Tumors often grow centrifugally and can compress surrounding normal structures. Infrequently, tumor impinge-ment on bone or neurovascular bundles produces pain, edema, and swelling. Less frequently, tumors cause obstructive gastro-intestinal symptoms or neurologic symptoms related to com-pression of lumbar or pelvic nerves. Often an extremity mass is discovered after a traumatic event that draws attention to a preexisting lesion.The differential diagnosis of a soft tissue mass should include consideration of lipoma (which is 100 times more com-mon than sarcoma), lymphangioma, leiomyoma, neurinoma, primary or |
Surgery_Schwartz_10345 | Surgery_Schwartz | lesion.The differential diagnosis of a soft tissue mass should include consideration of lipoma (which is 100 times more com-mon than sarcoma), lymphangioma, leiomyoma, neurinoma, primary or metastatic carcinoma, melanoma, and lymphoma. Superficial small lesions (<5 cm) that are new or that are not enlarging as indicated by clinical history can be observed. Figure 36-2. A 55-year-old man with a leiomyosarcoma involving the inferior vena cava. Note the displacement of the inferior vena cava to the right hemiabdomen adjacent to the liver (arrow).Enlarging masses and masses larger than 5 cm or deep to the fascia should be evaluated with a history, imaging, and biopsy.30Diagnostic ImagingDiagnostic imaging of the primary should be performed before any invasive procedure to avoid the possibility of soft tissue swelling or hemorrhage complicating the image interpretation. Pretreatment diagnostic imaging is helpful for defining the size and anatomic location of a tumor and its proximity to | Surgery_Schwartz. lesion.The differential diagnosis of a soft tissue mass should include consideration of lipoma (which is 100 times more com-mon than sarcoma), lymphangioma, leiomyoma, neurinoma, primary or metastatic carcinoma, melanoma, and lymphoma. Superficial small lesions (<5 cm) that are new or that are not enlarging as indicated by clinical history can be observed. Figure 36-2. A 55-year-old man with a leiomyosarcoma involving the inferior vena cava. Note the displacement of the inferior vena cava to the right hemiabdomen adjacent to the liver (arrow).Enlarging masses and masses larger than 5 cm or deep to the fascia should be evaluated with a history, imaging, and biopsy.30Diagnostic ImagingDiagnostic imaging of the primary should be performed before any invasive procedure to avoid the possibility of soft tissue swelling or hemorrhage complicating the image interpretation. Pretreatment diagnostic imaging is helpful for defining the size and anatomic location of a tumor and its proximity to |
Surgery_Schwartz_10346 | Surgery_Schwartz | of soft tissue swelling or hemorrhage complicating the image interpretation. Pretreatment diagnostic imaging is helpful for defining the size and anatomic location of a tumor and its proximity to adjacent structures; staging disease with respect to regional or metastatic spread; guiding percutaneous biopsy; and establishing whether a tumor is benign or malignant and low grade or high grade.Radiographs are useful in the evaluation of primary bone tumors but not in the evaluation of soft tissue sarcomas of the extremities unless there is underlying bone involvement from an adjacent soft tissue tumor or mineralization patterns sugges-tive of histologic subtype. Magnetic resonance imaging (MRI) is the preferred imaging technique for soft tissue sarcomas of the extremities, whereas computed tomography (CT) is most use-ful for evaluating retroperitoneal, intra-abdominal, and truncal sarcomas.31 CT of the chest should be performed to assess for lung metastases in patients with high-grade | Surgery_Schwartz. of soft tissue swelling or hemorrhage complicating the image interpretation. Pretreatment diagnostic imaging is helpful for defining the size and anatomic location of a tumor and its proximity to adjacent structures; staging disease with respect to regional or metastatic spread; guiding percutaneous biopsy; and establishing whether a tumor is benign or malignant and low grade or high grade.Radiographs are useful in the evaluation of primary bone tumors but not in the evaluation of soft tissue sarcomas of the extremities unless there is underlying bone involvement from an adjacent soft tissue tumor or mineralization patterns sugges-tive of histologic subtype. Magnetic resonance imaging (MRI) is the preferred imaging technique for soft tissue sarcomas of the extremities, whereas computed tomography (CT) is most use-ful for evaluating retroperitoneal, intra-abdominal, and truncal sarcomas.31 CT of the chest should be performed to assess for lung metastases in patients with high-grade |
Surgery_Schwartz_10347 | Surgery_Schwartz | tomography (CT) is most use-ful for evaluating retroperitoneal, intra-abdominal, and truncal sarcomas.31 CT of the chest should be performed to assess for lung metastases in patients with high-grade tumors larger than 5 cm; while chest X-ray is sufficient for smaller or low-grade lesions. Abdominal/pelvic CT should be performed in patients with myxoid round cell liposarcomas, leiomyosarcomas, epi-thelioid sarcomas, or angiosarcomas because of their propensity to metastasize to the abdomen and/or pelvis.1 Total Spine MRI has been advocated for myxoid round cell liposarcoma.1 MRI of the brain should be considered for patients with alveolar soft part sarcomas and angiosarcomas because of their propensity to metastasize to the brain.Ultrasonography. Ultrasonography may have a diagnostic role in patients with soft tissue sarcoma who cannot undergo MRI. Ultrasonography can also be a useful adjunct to MRI when findings on MRI are indeterminate and for delineating adjacent vascular | Surgery_Schwartz. tomography (CT) is most use-ful for evaluating retroperitoneal, intra-abdominal, and truncal sarcomas.31 CT of the chest should be performed to assess for lung metastases in patients with high-grade tumors larger than 5 cm; while chest X-ray is sufficient for smaller or low-grade lesions. Abdominal/pelvic CT should be performed in patients with myxoid round cell liposarcomas, leiomyosarcomas, epi-thelioid sarcomas, or angiosarcomas because of their propensity to metastasize to the abdomen and/or pelvis.1 Total Spine MRI has been advocated for myxoid round cell liposarcoma.1 MRI of the brain should be considered for patients with alveolar soft part sarcomas and angiosarcomas because of their propensity to metastasize to the brain.Ultrasonography. Ultrasonography may have a diagnostic role in patients with soft tissue sarcoma who cannot undergo MRI. Ultrasonography can also be a useful adjunct to MRI when findings on MRI are indeterminate and for delineating adjacent vascular |
Surgery_Schwartz_10348 | Surgery_Schwartz | role in patients with soft tissue sarcoma who cannot undergo MRI. Ultrasonography can also be a useful adjunct to MRI when findings on MRI are indeterminate and for delineating adjacent vascular structures. Finally, ultrasonography can be used for postoperative surveillance and to guide biopsies.Computed Tomography. Chest CT should be performed to evaluate for lung metastasis at presentation and before any radi-cal treatment. CT is also the preferred imaging technique for evaluating retroperitoneal sarcomas (Fig. 36-2).31 Current CT Brunicardi_Ch36_p1567-p1598.indd 157001/03/19 6:38 PM 1571SOFT TISSUE SARCOMASCHAPTER 36techniques can provide a detailed image of the abdomen and pelvis and can delineate adjacent organs and vascular struc-tures. For extremity sarcomas, CT may be useful if MRI is not available or cannot be used. When histologic assessment of an extremity sarcoma reveals a myxoid liposarcoma, CT of the abdomen and pelvis should be done because this subtype is known to | Surgery_Schwartz. role in patients with soft tissue sarcoma who cannot undergo MRI. Ultrasonography can also be a useful adjunct to MRI when findings on MRI are indeterminate and for delineating adjacent vascular structures. Finally, ultrasonography can be used for postoperative surveillance and to guide biopsies.Computed Tomography. Chest CT should be performed to evaluate for lung metastasis at presentation and before any radi-cal treatment. CT is also the preferred imaging technique for evaluating retroperitoneal sarcomas (Fig. 36-2).31 Current CT Brunicardi_Ch36_p1567-p1598.indd 157001/03/19 6:38 PM 1571SOFT TISSUE SARCOMASCHAPTER 36techniques can provide a detailed image of the abdomen and pelvis and can delineate adjacent organs and vascular struc-tures. For extremity sarcomas, CT may be useful if MRI is not available or cannot be used. When histologic assessment of an extremity sarcoma reveals a myxoid liposarcoma, CT of the abdomen and pelvis should be done because this subtype is known to |
Surgery_Schwartz_10349 | Surgery_Schwartz | MRI is not available or cannot be used. When histologic assessment of an extremity sarcoma reveals a myxoid liposarcoma, CT of the abdomen and pelvis should be done because this subtype is known to metastasize to the abdomen.32Magnetic Resonance Imaging. MRI is the most useful imag-ing modality for extremity sarcomas because of its superior soft tissue contrast resolution and multiplanar capabilities. MRI accurately delineates muscle groups and distinguishes among bone, vascular structures, and tumor. Sagittal and coronal views allow evaluation of anatomic compartments in three dimensions (Fig. 36-3). Soft tissue sarcomas of the extremities usually present on MRI as a heterogeneous mass. Their signal inten-sity tends to be equal to or slightly higher than that of adjacent skeletal muscle on T1-weighted images and heterogeneous and high on T2-weighted images. Hemorrhagic, cystic, or necrotic changes may also be observed in the tumor. If adjacent vas-cular structures must be delineated, | Surgery_Schwartz. MRI is not available or cannot be used. When histologic assessment of an extremity sarcoma reveals a myxoid liposarcoma, CT of the abdomen and pelvis should be done because this subtype is known to metastasize to the abdomen.32Magnetic Resonance Imaging. MRI is the most useful imag-ing modality for extremity sarcomas because of its superior soft tissue contrast resolution and multiplanar capabilities. MRI accurately delineates muscle groups and distinguishes among bone, vascular structures, and tumor. Sagittal and coronal views allow evaluation of anatomic compartments in three dimensions (Fig. 36-3). Soft tissue sarcomas of the extremities usually present on MRI as a heterogeneous mass. Their signal inten-sity tends to be equal to or slightly higher than that of adjacent skeletal muscle on T1-weighted images and heterogeneous and high on T2-weighted images. Hemorrhagic, cystic, or necrotic changes may also be observed in the tumor. If adjacent vas-cular structures must be delineated, |
Surgery_Schwartz_10350 | Surgery_Schwartz | T1-weighted images and heterogeneous and high on T2-weighted images. Hemorrhagic, cystic, or necrotic changes may also be observed in the tumor. If adjacent vas-cular structures must be delineated, special MRI techniques may be performed, including magnetic resonance angiography. MRI may also be an important adjunct to cytologic analysis in distinguishing benign lesions such as lipomas, hemangiomas, schwannomas, neurofibromas, and intramuscular myxomas from their malignant counterparts. In patients undergoing pre-operative chemotherapy, contrast-enhanced T1-weighted MRI can be useful in evaluating intratumoral necrosis.MRI is also valuable for assessing tumor recurrence after surgery. A baseline image is usually obtained 3 months after surgery. Some clinicians forego routine postoperative imaging of the primary extremity tumor site in asymptomatic patients, citing the difficulties in detecting early recurrence in scarred, irradiated tissue.31 Others advocate routine imaging every 3 to | Surgery_Schwartz. T1-weighted images and heterogeneous and high on T2-weighted images. Hemorrhagic, cystic, or necrotic changes may also be observed in the tumor. If adjacent vas-cular structures must be delineated, special MRI techniques may be performed, including magnetic resonance angiography. MRI may also be an important adjunct to cytologic analysis in distinguishing benign lesions such as lipomas, hemangiomas, schwannomas, neurofibromas, and intramuscular myxomas from their malignant counterparts. In patients undergoing pre-operative chemotherapy, contrast-enhanced T1-weighted MRI can be useful in evaluating intratumoral necrosis.MRI is also valuable for assessing tumor recurrence after surgery. A baseline image is usually obtained 3 months after surgery. Some clinicians forego routine postoperative imaging of the primary extremity tumor site in asymptomatic patients, citing the difficulties in detecting early recurrence in scarred, irradiated tissue.31 Others advocate routine imaging every 3 to |
Surgery_Schwartz_10351 | Surgery_Schwartz | imaging of the primary extremity tumor site in asymptomatic patients, citing the difficulties in detecting early recurrence in scarred, irradiated tissue.31 Others advocate routine imaging every 3 to 4 months for the first 2 years, every 6 months in years 3 through 5, and then annually.Positron Emission Tomography. Positron emission tomogra-phy (PET) is a functional imaging modality that measures tumor uptake of the glucose analog [18F] fluorodeoxyglucose (FDG). Figure 36-3. A 62-year-old man presented with right thigh mass. Magnetic resonance imaging demonstrated an 18 × 15 cm2 dedifferentiated liposarcoma within the posterior compartment. Note the atypical fatty mass (left) with a large necrotic and peripherally enhancing nodule (left).PET imaging allows evaluation of the entire body. Although PET/CT may be useful in specific circumstances, FDG-PET is not currently recommended for the initial staging of patients with soft tissue sarcoma.Roberge and colleagues compared FDG-PET/CT | Surgery_Schwartz. imaging of the primary extremity tumor site in asymptomatic patients, citing the difficulties in detecting early recurrence in scarred, irradiated tissue.31 Others advocate routine imaging every 3 to 4 months for the first 2 years, every 6 months in years 3 through 5, and then annually.Positron Emission Tomography. Positron emission tomogra-phy (PET) is a functional imaging modality that measures tumor uptake of the glucose analog [18F] fluorodeoxyglucose (FDG). Figure 36-3. A 62-year-old man presented with right thigh mass. Magnetic resonance imaging demonstrated an 18 × 15 cm2 dedifferentiated liposarcoma within the posterior compartment. Note the atypical fatty mass (left) with a large necrotic and peripherally enhancing nodule (left).PET imaging allows evaluation of the entire body. Although PET/CT may be useful in specific circumstances, FDG-PET is not currently recommended for the initial staging of patients with soft tissue sarcoma.Roberge and colleagues compared FDG-PET/CT |
Surgery_Schwartz_10352 | Surgery_Schwartz | Although PET/CT may be useful in specific circumstances, FDG-PET is not currently recommended for the initial staging of patients with soft tissue sarcoma.Roberge and colleagues compared FDG-PET/CT versus chest CT alone in the initial staging of 75 patients with soft tissue sarcoma and found that only one patient had disease upstaged as a result of PET, whereas two had false-positive find-ings and three had indeterminate findings with no subsequent development of metastasis.33 Previous studies that reported a marginal benefit of PET/CT for detecting metastasis at the time of sarcoma staging included patients with more heterogeneous tumors, such as osseous tumors, soft tissue osteosarcomas, Ewing’s sarcoma, and rhabdomyosarcoma.34-36In patients with sarcoma, PET has primarily been used to assist with tumor grading and to assess response to chemotherapy.37-40 In 50 patients with resectable high-grade soft tissue tumors scheduled for preoperative chemotherapy and tumor resection, a 35% | Surgery_Schwartz. Although PET/CT may be useful in specific circumstances, FDG-PET is not currently recommended for the initial staging of patients with soft tissue sarcoma.Roberge and colleagues compared FDG-PET/CT versus chest CT alone in the initial staging of 75 patients with soft tissue sarcoma and found that only one patient had disease upstaged as a result of PET, whereas two had false-positive find-ings and three had indeterminate findings with no subsequent development of metastasis.33 Previous studies that reported a marginal benefit of PET/CT for detecting metastasis at the time of sarcoma staging included patients with more heterogeneous tumors, such as osseous tumors, soft tissue osteosarcomas, Ewing’s sarcoma, and rhabdomyosarcoma.34-36In patients with sarcoma, PET has primarily been used to assist with tumor grading and to assess response to chemotherapy.37-40 In 50 patients with resectable high-grade soft tissue tumors scheduled for preoperative chemotherapy and tumor resection, a 35% |
Surgery_Schwartz_10353 | Surgery_Schwartz | with tumor grading and to assess response to chemotherapy.37-40 In 50 patients with resectable high-grade soft tissue tumors scheduled for preoperative chemotherapy and tumor resection, a 35% or greater reduction in tumor FDG uptake following an initial cycle of chemotherapy was associated with histopathologic tumor response defined as pathologic necrosis in 95% or more of the resected specimen.41 While this is helpful in determining response controversy exists as to whether this translates into a predictor of overall survival.Biopsy TechniquesFine-Needle Aspiration. At centers where cytopathologists have experience with evaluation of mesenchymal tumors, fine-needle aspiration is an acceptable method of diagnosing most soft tissue sarcomas, particularly when the results correlate closely with clinical and radiologic findings.42 Fine-needle aspiration of primary tumors has a lower diagnostic accuracy rate (60–90%) than core needle biopsy and is often not sufficient for establish-ing | Surgery_Schwartz. with tumor grading and to assess response to chemotherapy.37-40 In 50 patients with resectable high-grade soft tissue tumors scheduled for preoperative chemotherapy and tumor resection, a 35% or greater reduction in tumor FDG uptake following an initial cycle of chemotherapy was associated with histopathologic tumor response defined as pathologic necrosis in 95% or more of the resected specimen.41 While this is helpful in determining response controversy exists as to whether this translates into a predictor of overall survival.Biopsy TechniquesFine-Needle Aspiration. At centers where cytopathologists have experience with evaluation of mesenchymal tumors, fine-needle aspiration is an acceptable method of diagnosing most soft tissue sarcomas, particularly when the results correlate closely with clinical and radiologic findings.42 Fine-needle aspiration of primary tumors has a lower diagnostic accuracy rate (60–90%) than core needle biopsy and is often not sufficient for establish-ing |
Surgery_Schwartz_10354 | Surgery_Schwartz | with clinical and radiologic findings.42 Fine-needle aspiration of primary tumors has a lower diagnostic accuracy rate (60–90%) than core needle biopsy and is often not sufficient for establish-ing a specific histologic diagnosis and grade.43 However, fine-needle aspiration is the procedure of choice to confirm or rule out the presence of a metastatic focus or local recurrence.44Although fine-needle aspiration of superficial lesions can often be done in the clinic, fine-needle aspiration of deeper tumors may need to be done by an interventional radiologist Brunicardi_Ch36_p1567-p1598.indd 157101/03/19 6:38 PM 1572SPECIFIC CONSIDERATIONSPART IIunder sonographic or CT guidance. Generally, a 21to 23-gauge needle is introduced into the mass after appropriate cleansing of the skin and injection of local anesthetic. Negative pressure is applied, and the needle is moved back and forth several times in various directions. After the negative pressure is released, the needle is withdrawn, | Surgery_Schwartz. with clinical and radiologic findings.42 Fine-needle aspiration of primary tumors has a lower diagnostic accuracy rate (60–90%) than core needle biopsy and is often not sufficient for establish-ing a specific histologic diagnosis and grade.43 However, fine-needle aspiration is the procedure of choice to confirm or rule out the presence of a metastatic focus or local recurrence.44Although fine-needle aspiration of superficial lesions can often be done in the clinic, fine-needle aspiration of deeper tumors may need to be done by an interventional radiologist Brunicardi_Ch36_p1567-p1598.indd 157101/03/19 6:38 PM 1572SPECIFIC CONSIDERATIONSPART IIunder sonographic or CT guidance. Generally, a 21to 23-gauge needle is introduced into the mass after appropriate cleansing of the skin and injection of local anesthetic. Negative pressure is applied, and the needle is moved back and forth several times in various directions. After the negative pressure is released, the needle is withdrawn, |
Surgery_Schwartz_10355 | Surgery_Schwartz | of local anesthetic. Negative pressure is applied, and the needle is moved back and forth several times in various directions. After the negative pressure is released, the needle is withdrawn, and the contents of the needle are used to prepare smears.45 A cytopathologist then examines the slides to determine whether sufficient diagnostic material is present.Core Needle Biopsy. Core needle biopsy is safe, accurate,46,47 and economical48 and has become the preferred technique for diagnosing soft tissue lesions. Dupuy and colleagues found that core needle biopsy had an accuracy of 93% in 221 patients with musculoskeletal neoplasms.46Image guidance (ultrasound or CT) can prevent sam-pling of nondiagnostic necrotic or cystic areas of the tumor and thus increase the positive yield rate. Image guidance also per-mits biopsy of tumors in otherwise inaccessible locations and tumors located near vital structures with less risk of injury or complication.The tissue sample obtained from core needle | Surgery_Schwartz. of local anesthetic. Negative pressure is applied, and the needle is moved back and forth several times in various directions. After the negative pressure is released, the needle is withdrawn, and the contents of the needle are used to prepare smears.45 A cytopathologist then examines the slides to determine whether sufficient diagnostic material is present.Core Needle Biopsy. Core needle biopsy is safe, accurate,46,47 and economical48 and has become the preferred technique for diagnosing soft tissue lesions. Dupuy and colleagues found that core needle biopsy had an accuracy of 93% in 221 patients with musculoskeletal neoplasms.46Image guidance (ultrasound or CT) can prevent sam-pling of nondiagnostic necrotic or cystic areas of the tumor and thus increase the positive yield rate. Image guidance also per-mits biopsy of tumors in otherwise inaccessible locations and tumors located near vital structures with less risk of injury or complication.The tissue sample obtained from core needle |
Surgery_Schwartz_10356 | Surgery_Schwartz | also per-mits biopsy of tumors in otherwise inaccessible locations and tumors located near vital structures with less risk of injury or complication.The tissue sample obtained from core needle biopsy is usually sufficient for several diagnostic tests, such as electron microscopy, cytogenetic analysis, and flow cytometry. The risk for needle track seeding is negligible, and the reported compli-cation rate for core needle biopsy is less than 1%.46,47Incisional Biopsy. Historically, an open surgical biopsy was the gold standard for achieving adequate tissue for definitive and specific histologic diagnosis of bone or soft tissue sarcomas. Contemporary guidelines recommend incisional biopsy when core needle biopsy cannot produce adequate tissue for diagnosis or when findings on core needle biopsy are nondiagnostic.The disadvantages of incisional biopsy include the need to schedule the procedure, the need for general anesthesia, and high costs. In addition, an inappropriately placed | Surgery_Schwartz. also per-mits biopsy of tumors in otherwise inaccessible locations and tumors located near vital structures with less risk of injury or complication.The tissue sample obtained from core needle biopsy is usually sufficient for several diagnostic tests, such as electron microscopy, cytogenetic analysis, and flow cytometry. The risk for needle track seeding is negligible, and the reported compli-cation rate for core needle biopsy is less than 1%.46,47Incisional Biopsy. Historically, an open surgical biopsy was the gold standard for achieving adequate tissue for definitive and specific histologic diagnosis of bone or soft tissue sarcomas. Contemporary guidelines recommend incisional biopsy when core needle biopsy cannot produce adequate tissue for diagnosis or when findings on core needle biopsy are nondiagnostic.The disadvantages of incisional biopsy include the need to schedule the procedure, the need for general anesthesia, and high costs. In addition, an inappropriately placed |
Surgery_Schwartz_10357 | Surgery_Schwartz | biopsy are nondiagnostic.The disadvantages of incisional biopsy include the need to schedule the procedure, the need for general anesthesia, and high costs. In addition, an inappropriately placed incision can necessitate more extensive definitive resection to incorporate the biopsy incision. In a series of 107 patients with soft tissue sarcoma, planned surgical treatments had to be changed because of poorly oriented biopsies in 25% of cases.49 Complication rates up to 17% have been reported after incisional biopsies.45 Potential complications include hematoma, infection, wound dehiscence, and tumor fungation, any of which can delay defini-tive treatment.45Incisional biopsies should be performed only by surgeons experienced in the management of soft tissue sarcoma, ideally in a center specializing in the treatment of sarcoma and by the surgeon who will perform the definitive surgery. The biopsy incision should be oriented longitudinally along the extremity to allow a subsequent wide | Surgery_Schwartz. biopsy are nondiagnostic.The disadvantages of incisional biopsy include the need to schedule the procedure, the need for general anesthesia, and high costs. In addition, an inappropriately placed incision can necessitate more extensive definitive resection to incorporate the biopsy incision. In a series of 107 patients with soft tissue sarcoma, planned surgical treatments had to be changed because of poorly oriented biopsies in 25% of cases.49 Complication rates up to 17% have been reported after incisional biopsies.45 Potential complications include hematoma, infection, wound dehiscence, and tumor fungation, any of which can delay defini-tive treatment.45Incisional biopsies should be performed only by surgeons experienced in the management of soft tissue sarcoma, ideally in a center specializing in the treatment of sarcoma and by the surgeon who will perform the definitive surgery. The biopsy incision should be oriented longitudinally along the extremity to allow a subsequent wide |
Surgery_Schwartz_10358 | Surgery_Schwartz | in the treatment of sarcoma and by the surgeon who will perform the definitive surgery. The biopsy incision should be oriented longitudinally along the extremity to allow a subsequent wide local excision that encompasses the biopsy site, scar, and tumor en bloc minimizing the risk for increasing the complexity of the subsequent curative procedure. A poorly oriented biopsy incision often necessitates an exces-sively large surgical defect for a wide local excision, which in turn can result in a larger postoperative radiation therapy field to encompass all tissues at risk. Adequate hemostasis must be achieved at the time of biopsy to prevent dissemination of tumor cells into adjacent tissue planes by hematoma.Excisional Biopsy. Excisional biopsy can be performed for easily accessible (superficial) extremity or truncal lesions smaller than 3 cm. However, excisional biopsy rarely provides benefits over other biopsy techniques. Excisional biopsies should not be performed for lesions | Surgery_Schwartz. in the treatment of sarcoma and by the surgeon who will perform the definitive surgery. The biopsy incision should be oriented longitudinally along the extremity to allow a subsequent wide local excision that encompasses the biopsy site, scar, and tumor en bloc minimizing the risk for increasing the complexity of the subsequent curative procedure. A poorly oriented biopsy incision often necessitates an exces-sively large surgical defect for a wide local excision, which in turn can result in a larger postoperative radiation therapy field to encompass all tissues at risk. Adequate hemostasis must be achieved at the time of biopsy to prevent dissemination of tumor cells into adjacent tissue planes by hematoma.Excisional Biopsy. Excisional biopsy can be performed for easily accessible (superficial) extremity or truncal lesions smaller than 3 cm. However, excisional biopsy rarely provides benefits over other biopsy techniques. Excisional biopsies should not be performed for lesions |
Surgery_Schwartz_10359 | Surgery_Schwartz | (superficial) extremity or truncal lesions smaller than 3 cm. However, excisional biopsy rarely provides benefits over other biopsy techniques. Excisional biopsies should not be performed for lesions involving the hands and feet because such biopsies may complicate definitive reexcision. For sarcomas with initial diagnosis confirmed with excisional biopsy, microscopic residual disease has been reported in up to 69% of reexcision specimens50,51; without reexcision, the reported rate of local recurrence is 30% to 40% when margins are positive or uncertain.Wide en bloc excision is seldom performed as a diagnostic procedure. When en bloc excision is done for diagnosis, the mar-gin status is often not adequately evaluated during pathologic assessment of the specimen. Unless detailed descriptions of the surgical procedure and the pathology specimen are provided, the margins should be classified as uncertain or unknown, a classifi-cation associated with the same prognosis as resection | Surgery_Schwartz. (superficial) extremity or truncal lesions smaller than 3 cm. However, excisional biopsy rarely provides benefits over other biopsy techniques. Excisional biopsies should not be performed for lesions involving the hands and feet because such biopsies may complicate definitive reexcision. For sarcomas with initial diagnosis confirmed with excisional biopsy, microscopic residual disease has been reported in up to 69% of reexcision specimens50,51; without reexcision, the reported rate of local recurrence is 30% to 40% when margins are positive or uncertain.Wide en bloc excision is seldom performed as a diagnostic procedure. When en bloc excision is done for diagnosis, the mar-gin status is often not adequately evaluated during pathologic assessment of the specimen. Unless detailed descriptions of the surgical procedure and the pathology specimen are provided, the margins should be classified as uncertain or unknown, a classifi-cation associated with the same prognosis as resection |
Surgery_Schwartz_10360 | Surgery_Schwartz | of the surgical procedure and the pathology specimen are provided, the margins should be classified as uncertain or unknown, a classifi-cation associated with the same prognosis as resection margins that are positive for tumor cells. In patients with uncertain or unknown margins, reexcision should be performed if possible to ensure negative margins. The biopsy site or tract (if applicable) should be included en bloc with the re-resected specimen.Pathologic Assessment and ClassificationSarcoma is generally diagnosed by morphologic assessment based on microscopic examination of histologic sections by an experienced sarcoma pathologist. However, even expert sar-coma pathologists disagree on the specific histologic diagnosis and the tumor grade in 25% to 40% of cases.52Morphologic assessment can be supported by ancillary techniques, including conventional cytogenetics; immunohis-tochemistry; and molecular genetic testing, which is useful for classifying soft tissue sarcoma subtypes with | Surgery_Schwartz. of the surgical procedure and the pathology specimen are provided, the margins should be classified as uncertain or unknown, a classifi-cation associated with the same prognosis as resection margins that are positive for tumor cells. In patients with uncertain or unknown margins, reexcision should be performed if possible to ensure negative margins. The biopsy site or tract (if applicable) should be included en bloc with the re-resected specimen.Pathologic Assessment and ClassificationSarcoma is generally diagnosed by morphologic assessment based on microscopic examination of histologic sections by an experienced sarcoma pathologist. However, even expert sar-coma pathologists disagree on the specific histologic diagnosis and the tumor grade in 25% to 40% of cases.52Morphologic assessment can be supported by ancillary techniques, including conventional cytogenetics; immunohis-tochemistry; and molecular genetic testing, which is useful for classifying soft tissue sarcoma subtypes with |
Surgery_Schwartz_10361 | Surgery_Schwartz | can be supported by ancillary techniques, including conventional cytogenetics; immunohis-tochemistry; and molecular genetic testing, which is useful for classifying soft tissue sarcoma subtypes with multiple genetic aberrations. Other molecular diagnostic techniques include cytogenetic analysis, fluorescence in situ hybridization, and polymerase chain reaction–based methods.53 However, molecu-lar genetic techniques are associated with significant technical limitations and should be interpreted in the context of the sar-coma’s morphologic features.Some experts have suggested that pathologic classification of soft tissue sarcomas has more prognostic significance than does tumor grade when other pretreatment variables are taken into account. Tumors with limited metastatic potential include des-moid, atypical lipomatous tumor (also called well-differentiated liposarcoma), dermatofibrosarcoma protuberans, and solitary fibrous tumor. Tumors with an intermediate risk of metastatic spread | Surgery_Schwartz. can be supported by ancillary techniques, including conventional cytogenetics; immunohis-tochemistry; and molecular genetic testing, which is useful for classifying soft tissue sarcoma subtypes with multiple genetic aberrations. Other molecular diagnostic techniques include cytogenetic analysis, fluorescence in situ hybridization, and polymerase chain reaction–based methods.53 However, molecu-lar genetic techniques are associated with significant technical limitations and should be interpreted in the context of the sar-coma’s morphologic features.Some experts have suggested that pathologic classification of soft tissue sarcomas has more prognostic significance than does tumor grade when other pretreatment variables are taken into account. Tumors with limited metastatic potential include des-moid, atypical lipomatous tumor (also called well-differentiated liposarcoma), dermatofibrosarcoma protuberans, and solitary fibrous tumor. Tumors with an intermediate risk of metastatic spread |
Surgery_Schwartz_10362 | Surgery_Schwartz | des-moid, atypical lipomatous tumor (also called well-differentiated liposarcoma), dermatofibrosarcoma protuberans, and solitary fibrous tumor. Tumors with an intermediate risk of metastatic spread usually have a large myxoid component and include myx-oid liposarcoma, myxofibrosarcoma, and extraskeletal myxoid chondrosarcoma. Among the highly aggressive tumors with sub-stantial metastatic potential are angiosarcoma, clear cell sarcoma, pleomorphic and dedifferentiated liposarcoma, leiomyosarcoma, MPNST, rhabdomyosarcoma, and synovial sarcoma.It has recently been noted that malignant fibrous histio-cytoma is not associated with a distinct gene cluster, suggest-ing that malignant fibrous histiocytoma is not a separate tumor entity but rather a common morphologic appearance of various sarcoma subtypes.54,55 For example, most tumors initially diag-nosed as malignant fibrous histiocytoma in the retroperitoneum have been reclassified using genomic profiling as dedifferenti-ated | Surgery_Schwartz. des-moid, atypical lipomatous tumor (also called well-differentiated liposarcoma), dermatofibrosarcoma protuberans, and solitary fibrous tumor. Tumors with an intermediate risk of metastatic spread usually have a large myxoid component and include myx-oid liposarcoma, myxofibrosarcoma, and extraskeletal myxoid chondrosarcoma. Among the highly aggressive tumors with sub-stantial metastatic potential are angiosarcoma, clear cell sarcoma, pleomorphic and dedifferentiated liposarcoma, leiomyosarcoma, MPNST, rhabdomyosarcoma, and synovial sarcoma.It has recently been noted that malignant fibrous histio-cytoma is not associated with a distinct gene cluster, suggest-ing that malignant fibrous histiocytoma is not a separate tumor entity but rather a common morphologic appearance of various sarcoma subtypes.54,55 For example, most tumors initially diag-nosed as malignant fibrous histiocytoma in the retroperitoneum have been reclassified using genomic profiling as dedifferenti-ated |
Surgery_Schwartz_10363 | Surgery_Schwartz | sarcoma subtypes.54,55 For example, most tumors initially diag-nosed as malignant fibrous histiocytoma in the retroperitoneum have been reclassified using genomic profiling as dedifferenti-ated liposarcomas,56 whereas those in the extremities have been reclassified as leiomyosarcoma, myxofibrosarcoma, or pleo-morphic undifferentiated sarcoma.Guidelines for the pathologic reporting of sarcoma have been established.1 Included in the report should be the primary diagnosis, anatomic site, depth, size, and histologic grade, pres-ence or absence of necrosis, status of excision margins and lymph nodes, TNM stage, and additional features of the tumor (i.e., mitotic rate and presence or absence of vascular invasion).Brunicardi_Ch36_p1567-p1598.indd 157201/03/19 6:38 PM 1573SOFT TISSUE SARCOMASCHAPTER 36Staging and Prognostic FactorsSoft tissue sarcoma is most commonly staged using either the American Joint Committee on Cancer (AJCC) system (generally used in the United States) or the World | Surgery_Schwartz. sarcoma subtypes.54,55 For example, most tumors initially diag-nosed as malignant fibrous histiocytoma in the retroperitoneum have been reclassified using genomic profiling as dedifferenti-ated liposarcomas,56 whereas those in the extremities have been reclassified as leiomyosarcoma, myxofibrosarcoma, or pleo-morphic undifferentiated sarcoma.Guidelines for the pathologic reporting of sarcoma have been established.1 Included in the report should be the primary diagnosis, anatomic site, depth, size, and histologic grade, pres-ence or absence of necrosis, status of excision margins and lymph nodes, TNM stage, and additional features of the tumor (i.e., mitotic rate and presence or absence of vascular invasion).Brunicardi_Ch36_p1567-p1598.indd 157201/03/19 6:38 PM 1573SOFT TISSUE SARCOMASCHAPTER 36Staging and Prognostic FactorsSoft tissue sarcoma is most commonly staged using either the American Joint Committee on Cancer (AJCC) system (generally used in the United States) or the World |
Surgery_Schwartz_10364 | Surgery_Schwartz | 36Staging and Prognostic FactorsSoft tissue sarcoma is most commonly staged using either the American Joint Committee on Cancer (AJCC) system (generally used in the United States) or the World Health Organization sys-tem. A unique aspect of sarcoma staging is the inclusion of tumor grade, which is one of the most important prognostic factors.57The seventh edition of the AJCC staging system for soft tissue sarcomas is based on histologic grade of aggressive-ness, tumor size and depth, and the presence of nodal or distant metastases.58 This system does not apply to GIST, fibromatosis (desmoid tumor), Kaposi’s sarcoma, or infantile fibrosarcoma.Histologic Grade of Aggressiveness. Histologic grade is the most important prognostic factor for patients with soft tissue sarcoma. For accurate determination of grade, an adequate tis-sue sample must be appropriately fixed, stained, and reviewed by an experienced sarcoma pathologist. The features that define grade are cellularity, differentiation | Surgery_Schwartz. 36Staging and Prognostic FactorsSoft tissue sarcoma is most commonly staged using either the American Joint Committee on Cancer (AJCC) system (generally used in the United States) or the World Health Organization sys-tem. A unique aspect of sarcoma staging is the inclusion of tumor grade, which is one of the most important prognostic factors.57The seventh edition of the AJCC staging system for soft tissue sarcomas is based on histologic grade of aggressive-ness, tumor size and depth, and the presence of nodal or distant metastases.58 This system does not apply to GIST, fibromatosis (desmoid tumor), Kaposi’s sarcoma, or infantile fibrosarcoma.Histologic Grade of Aggressiveness. Histologic grade is the most important prognostic factor for patients with soft tissue sarcoma. For accurate determination of grade, an adequate tis-sue sample must be appropriately fixed, stained, and reviewed by an experienced sarcoma pathologist. The features that define grade are cellularity, differentiation |
Surgery_Schwartz_10365 | Surgery_Schwartz | of grade, an adequate tis-sue sample must be appropriately fixed, stained, and reviewed by an experienced sarcoma pathologist. The features that define grade are cellularity, differentiation (good, moderate, or poor/anaplastic), pleomorphism, necrosis (absent, <50%, or ≥50%), and number of mitoses per high-power field (<10, 10–19, or ≥20). Tumor grade has been shown to predict metastasis and overall survival.59 Metastasis has been estimated to occur in 5% to 10% of low-grade lesions, 25% to 30% of intermediate-grade lesions, and 50% to 60% of high-grade lesions.The number of grades varies according to the classifica-tion system used. The most common classification systems, those of the National Cancer Institute and the French Federation of Cancer Centers, use three-tier tumor grades.60 The National Cancer Institute system is based primarily on histologic sub-type, location, and amount of necrosis. The French Federation of Cancer Centers system is based on tumor differentiation (good, | Surgery_Schwartz. of grade, an adequate tis-sue sample must be appropriately fixed, stained, and reviewed by an experienced sarcoma pathologist. The features that define grade are cellularity, differentiation (good, moderate, or poor/anaplastic), pleomorphism, necrosis (absent, <50%, or ≥50%), and number of mitoses per high-power field (<10, 10–19, or ≥20). Tumor grade has been shown to predict metastasis and overall survival.59 Metastasis has been estimated to occur in 5% to 10% of low-grade lesions, 25% to 30% of intermediate-grade lesions, and 50% to 60% of high-grade lesions.The number of grades varies according to the classifica-tion system used. The most common classification systems, those of the National Cancer Institute and the French Federation of Cancer Centers, use three-tier tumor grades.60 The National Cancer Institute system is based primarily on histologic sub-type, location, and amount of necrosis. The French Federation of Cancer Centers system is based on tumor differentiation (good, |
Surgery_Schwartz_10366 | Surgery_Schwartz | National Cancer Institute system is based primarily on histologic sub-type, location, and amount of necrosis. The French Federation of Cancer Centers system is based on tumor differentiation (good, moderate, or poor/anaplastic), number of mitoses per high-power field (<10, 10–19, or ≥20), and amount of tumor necrosis (absent, <50%, or ≥50%). A comparative analysis of the two systems suggested that the French Federation of Can-cer Centers system has better prognostic capability, predicting 5-year survival rates of 90%, 70%, and 40% for grade 1, 2, and 3 tumors, respectively.60Following the recommendation of the College of American Pathologists, the committee that developed the 2008 AJCC staging system changed the system from a four-grade to a three-grade system in which the grades are well differentiated (grade 1), moderately differentiated (grade 2), and poorly differentiated (grade 3).61 Grade 1 is considered low grade, and grades 2 and 3 are considered high grade.Tumor Size and | Surgery_Schwartz. National Cancer Institute system is based primarily on histologic sub-type, location, and amount of necrosis. The French Federation of Cancer Centers system is based on tumor differentiation (good, moderate, or poor/anaplastic), number of mitoses per high-power field (<10, 10–19, or ≥20), and amount of tumor necrosis (absent, <50%, or ≥50%). A comparative analysis of the two systems suggested that the French Federation of Can-cer Centers system has better prognostic capability, predicting 5-year survival rates of 90%, 70%, and 40% for grade 1, 2, and 3 tumors, respectively.60Following the recommendation of the College of American Pathologists, the committee that developed the 2008 AJCC staging system changed the system from a four-grade to a three-grade system in which the grades are well differentiated (grade 1), moderately differentiated (grade 2), and poorly differentiated (grade 3).61 Grade 1 is considered low grade, and grades 2 and 3 are considered high grade.Tumor Size and |
Surgery_Schwartz_10367 | Surgery_Schwartz | differentiated (grade 1), moderately differentiated (grade 2), and poorly differentiated (grade 3).61 Grade 1 is considered low grade, and grades 2 and 3 are considered high grade.Tumor Size and Location. Tumor size is an important prog-nostic variable in soft tissue sarcomas. Sarcomas have classi-cally been stratified into two size groups; T1 lesions are 5 cm or smaller, and T2 lesions are larger than 5 cm. The staging of soft tissue sarcomas has changed considerably in the eighth edition of the AJCC Cancer Staging Handbook.62 Previously, all soft tissue sarcoma sites were staged as one. In the new edi-tion, subsites have been created, and they include head and neck; extremity and trunk; gastrointestinal tract; genitourinary tract; viscera and peritoneum; gynecological sites; breast lung, pleura and mediastinum; and other histologies. Size criteria for head and neck tumors have been reduced from the classic cri-teria because of a worse prognosis at smaller sizes. Trunk and extremity | Surgery_Schwartz. differentiated (grade 1), moderately differentiated (grade 2), and poorly differentiated (grade 3).61 Grade 1 is considered low grade, and grades 2 and 3 are considered high grade.Tumor Size and Location. Tumor size is an important prog-nostic variable in soft tissue sarcomas. Sarcomas have classi-cally been stratified into two size groups; T1 lesions are 5 cm or smaller, and T2 lesions are larger than 5 cm. The staging of soft tissue sarcomas has changed considerably in the eighth edition of the AJCC Cancer Staging Handbook.62 Previously, all soft tissue sarcoma sites were staged as one. In the new edi-tion, subsites have been created, and they include head and neck; extremity and trunk; gastrointestinal tract; genitourinary tract; viscera and peritoneum; gynecological sites; breast lung, pleura and mediastinum; and other histologies. Size criteria for head and neck tumors have been reduced from the classic cri-teria because of a worse prognosis at smaller sizes. Trunk and extremity |
Surgery_Schwartz_10368 | Surgery_Schwartz | pleura and mediastinum; and other histologies. Size criteria for head and neck tumors have been reduced from the classic cri-teria because of a worse prognosis at smaller sizes. Trunk and extremity sarcoma has been reclassified relative to size criteria for tumor stage, changing the four categories: T1 tumors are smaller than 5 cm; T2 tumors are 5 to 10 cm; T3 tumors are 10 to 15 cm; and T4 tumors are larger than 15 cm.62Figure 36-4. A 74-year-old man with a history of an extraskeletal myxoid chondrosarcoma of the gluteal region developed multiple lung metastases.Anatomic tumor location was incorporated into the AJCC staging system in 1998. Soft tissue sarcomas above the superfi-cial investing fascia of the extremity or trunk are designated “a” lesions within the T category, whereas tumors invading or deep to the fascia and all retroperitoneal, mediastinal, and visceral tumors are designated “b” lesions. However, in this most recent AJCC staging system, the superficial versus deep | Surgery_Schwartz. pleura and mediastinum; and other histologies. Size criteria for head and neck tumors have been reduced from the classic cri-teria because of a worse prognosis at smaller sizes. Trunk and extremity sarcoma has been reclassified relative to size criteria for tumor stage, changing the four categories: T1 tumors are smaller than 5 cm; T2 tumors are 5 to 10 cm; T3 tumors are 10 to 15 cm; and T4 tumors are larger than 15 cm.62Figure 36-4. A 74-year-old man with a history of an extraskeletal myxoid chondrosarcoma of the gluteal region developed multiple lung metastases.Anatomic tumor location was incorporated into the AJCC staging system in 1998. Soft tissue sarcomas above the superfi-cial investing fascia of the extremity or trunk are designated “a” lesions within the T category, whereas tumors invading or deep to the fascia and all retroperitoneal, mediastinal, and visceral tumors are designated “b” lesions. However, in this most recent AJCC staging system, the superficial versus deep |
Surgery_Schwartz_10369 | Surgery_Schwartz | invading or deep to the fascia and all retroperitoneal, mediastinal, and visceral tumors are designated “b” lesions. However, in this most recent AJCC staging system, the superficial versus deep distinction is less important and has been eliminated.62Nodal Metastasis. Overall, lymph node metastases arising from soft tissue sarcomas are rare,28 but the incidence of nodal involvement is higher for epithelioid sarcoma, pediatric rhab-domyosarcoma, clear cell sarcoma, synovial sarcoma, myxo-fibrosarcoma, and angiosarcoma. In the seventh edition of the AJCC staging system, sarcoma associated with nodal metas-tases was reclassified as stage III rather than stage IV because several studies reported better survival for patients with isolated regional lymph node metastases treated with radical lymphad-enectomy than for patients with distant metastases.28,63-65 How-ever, in the eighth edition of the AJCC, nodal disease has been revisited: N1 disease behaves similarly between stages III and IV | Surgery_Schwartz. invading or deep to the fascia and all retroperitoneal, mediastinal, and visceral tumors are designated “b” lesions. However, in this most recent AJCC staging system, the superficial versus deep distinction is less important and has been eliminated.62Nodal Metastasis. Overall, lymph node metastases arising from soft tissue sarcomas are rare,28 but the incidence of nodal involvement is higher for epithelioid sarcoma, pediatric rhab-domyosarcoma, clear cell sarcoma, synovial sarcoma, myxo-fibrosarcoma, and angiosarcoma. In the seventh edition of the AJCC staging system, sarcoma associated with nodal metas-tases was reclassified as stage III rather than stage IV because several studies reported better survival for patients with isolated regional lymph node metastases treated with radical lymphad-enectomy than for patients with distant metastases.28,63-65 How-ever, in the eighth edition of the AJCC, nodal disease has been revisited: N1 disease behaves similarly between stages III and IV |
Surgery_Schwartz_10370 | Surgery_Schwartz | than for patients with distant metastases.28,63-65 How-ever, in the eighth edition of the AJCC, nodal disease has been revisited: N1 disease behaves similarly between stages III and IV and is now captured as stage IV.62 Patients with clinically or radiologically suspicious regional nodes should have metastases confirmed or ruled out by either fine-needle aspiration or core biopsy before radical lymphadenectomy.Distant Metastasis. Distant metastases occur most often in the lungs (Fig. 36-4). Selected patients with pulmonary metas-tases may survive for long periods after surgical resection and chemotherapy. Other potential sites of metastasis include bone (Fig. 36-5), brain (Fig. 36-6), and liver (Fig. 36-7). Visceral and retroperitoneal sarcomas have a higher incidence of liver and peritoneal metastases.Prognostic Factors. Prognostic variables in soft tissue sar-coma include primary tumor size, grade, and depth, all of which are incorporated into the staging system, as well as | Surgery_Schwartz. than for patients with distant metastases.28,63-65 How-ever, in the eighth edition of the AJCC, nodal disease has been revisited: N1 disease behaves similarly between stages III and IV and is now captured as stage IV.62 Patients with clinically or radiologically suspicious regional nodes should have metastases confirmed or ruled out by either fine-needle aspiration or core biopsy before radical lymphadenectomy.Distant Metastasis. Distant metastases occur most often in the lungs (Fig. 36-4). Selected patients with pulmonary metas-tases may survive for long periods after surgical resection and chemotherapy. Other potential sites of metastasis include bone (Fig. 36-5), brain (Fig. 36-6), and liver (Fig. 36-7). Visceral and retroperitoneal sarcomas have a higher incidence of liver and peritoneal metastases.Prognostic Factors. Prognostic variables in soft tissue sar-coma include primary tumor size, grade, and depth, all of which are incorporated into the staging system, as well as |
Surgery_Schwartz_10371 | Surgery_Schwartz | peritoneal metastases.Prognostic Factors. Prognostic variables in soft tissue sar-coma include primary tumor size, grade, and depth, all of which are incorporated into the staging system, as well as histology, Brunicardi_Ch36_p1567-p1598.indd 157301/03/19 6:38 PM 1574SPECIFIC CONSIDERATIONSPART IIFigure 36-5. A 36-year-old woman with history of multifocal sclerosing osteosarcoma of the humerus developed diffuse bony metastases 2 years after diagnosis.Figure 36-6. A 64-year-old man with a history of a T2 high-grade pleomorphic sarcoma of the thigh who developed brain metastases 14 months after diagnosis.Figure 36-7. A 33-year-old woman with osteosarcoma of the sternum who presented with a liver metastasis 3 years after diagnosis.tumor site, and presentation (local recurrence or initial diagno-sis). Patient factors such as older age and gender have also been associated with recurrence and mortality in several studies.66 A positive microscopic margin and early recurrence after | Surgery_Schwartz. peritoneal metastases.Prognostic Factors. Prognostic variables in soft tissue sar-coma include primary tumor size, grade, and depth, all of which are incorporated into the staging system, as well as histology, Brunicardi_Ch36_p1567-p1598.indd 157301/03/19 6:38 PM 1574SPECIFIC CONSIDERATIONSPART IIFigure 36-5. A 36-year-old woman with history of multifocal sclerosing osteosarcoma of the humerus developed diffuse bony metastases 2 years after diagnosis.Figure 36-6. A 64-year-old man with a history of a T2 high-grade pleomorphic sarcoma of the thigh who developed brain metastases 14 months after diagnosis.Figure 36-7. A 33-year-old woman with osteosarcoma of the sternum who presented with a liver metastasis 3 years after diagnosis.tumor site, and presentation (local recurrence or initial diagno-sis). Patient factors such as older age and gender have also been associated with recurrence and mortality in several studies.66 A positive microscopic margin and early recurrence after |
Surgery_Schwartz_10372 | Surgery_Schwartz | diagno-sis). Patient factors such as older age and gender have also been associated with recurrence and mortality in several studies.66 A positive microscopic margin and early recurrence after resection of an extremity sarcoma have been shown to be associated with decreased survival.67Several groups have reported that Ki-67, a proliferation marker, is correlated with a poor clinical outcome in high-grade extremity sarcomas.68,69 E-cadherin and catenins, proteins essen-tial for intercellular junctions, have been associated with poor outcome in patients with soft tissue sarcoma.68 Similarly, higher CD100 expression has been shown to correlate with higher pro-liferative potential and poorer outcome.69Prognostic Nomograms. Prognostic nomograms for soft tis-sue sarcoma have been introduced for use in patient counsel-ing, selecting appropriate surveillance strategies, and selecting patients for clinical trials.70 One such nomogram, developed by Kattan and colleagues at Memorial | Surgery_Schwartz. diagno-sis). Patient factors such as older age and gender have also been associated with recurrence and mortality in several studies.66 A positive microscopic margin and early recurrence after resection of an extremity sarcoma have been shown to be associated with decreased survival.67Several groups have reported that Ki-67, a proliferation marker, is correlated with a poor clinical outcome in high-grade extremity sarcomas.68,69 E-cadherin and catenins, proteins essen-tial for intercellular junctions, have been associated with poor outcome in patients with soft tissue sarcoma.68 Similarly, higher CD100 expression has been shown to correlate with higher pro-liferative potential and poorer outcome.69Prognostic Nomograms. Prognostic nomograms for soft tis-sue sarcoma have been introduced for use in patient counsel-ing, selecting appropriate surveillance strategies, and selecting patients for clinical trials.70 One such nomogram, developed by Kattan and colleagues at Memorial |
Surgery_Schwartz_10373 | Surgery_Schwartz | for use in patient counsel-ing, selecting appropriate surveillance strategies, and selecting patients for clinical trials.70 One such nomogram, developed by Kattan and colleagues at Memorial Sloan-Kettering Cancer Center, considers age, histology, grade, location, depth, and size to determine the likelihood of 12-year sarcoma-specific survival.70 Two validation studies using the nomogram demon-strated good predictive value.71 More recently, the same group of investigators developed histology subtype-specific nomo-grams for patients with liposarcoma, synovial sarcoma, and GIST72 and demonstrated that they were accurate in predicting disease-specific survival. Other investigators have just devel-oped a site-specific nomogram for patients with retroperitoneal sarcoma, demonstrating an accurate prediction of survival and disease recurrence.73TREATMENT OF EXTREMITY AND TRUNK WALL SARCOMAThe goals of treatment of soft tissue sarcoma are to maxi-mize the likelihood of long-term | Surgery_Schwartz. for use in patient counsel-ing, selecting appropriate surveillance strategies, and selecting patients for clinical trials.70 One such nomogram, developed by Kattan and colleagues at Memorial Sloan-Kettering Cancer Center, considers age, histology, grade, location, depth, and size to determine the likelihood of 12-year sarcoma-specific survival.70 Two validation studies using the nomogram demon-strated good predictive value.71 More recently, the same group of investigators developed histology subtype-specific nomo-grams for patients with liposarcoma, synovial sarcoma, and GIST72 and demonstrated that they were accurate in predicting disease-specific survival. Other investigators have just devel-oped a site-specific nomogram for patients with retroperitoneal sarcoma, demonstrating an accurate prediction of survival and disease recurrence.73TREATMENT OF EXTREMITY AND TRUNK WALL SARCOMAThe goals of treatment of soft tissue sarcoma are to maxi-mize the likelihood of long-term |
Surgery_Schwartz_10374 | Surgery_Schwartz | an accurate prediction of survival and disease recurrence.73TREATMENT OF EXTREMITY AND TRUNK WALL SARCOMAThe goals of treatment of soft tissue sarcoma are to maxi-mize the likelihood of long-term recurrence-free survival while minimizing morbidity and maximizing function. In the past two decades, a multimodality treatment approach with optimal sequencing of treatments for individual patients has been shown to improve survival.74 Furthermore, patients with soft tissue sarcoma treated at high-volume centers have been shown to have improved survival and functional outcomes.75 Care at such centers is particularly important for patients with high-risk and advanced disease.The overall 5-year survival rate for patients with all stages of soft tissue sarcoma is 50% to 60%. For patients with extremity sarcomas, a multidisciplinary treatment approach has resulted in local control rates exceeding 90% and 5-year survival rates exceeding 70%. Most patients who die of soft tissue sarcoma die of | Surgery_Schwartz. an accurate prediction of survival and disease recurrence.73TREATMENT OF EXTREMITY AND TRUNK WALL SARCOMAThe goals of treatment of soft tissue sarcoma are to maxi-mize the likelihood of long-term recurrence-free survival while minimizing morbidity and maximizing function. In the past two decades, a multimodality treatment approach with optimal sequencing of treatments for individual patients has been shown to improve survival.74 Furthermore, patients with soft tissue sarcoma treated at high-volume centers have been shown to have improved survival and functional outcomes.75 Care at such centers is particularly important for patients with high-risk and advanced disease.The overall 5-year survival rate for patients with all stages of soft tissue sarcoma is 50% to 60%. For patients with extremity sarcomas, a multidisciplinary treatment approach has resulted in local control rates exceeding 90% and 5-year survival rates exceeding 70%. Most patients who die of soft tissue sarcoma die of |
Surgery_Schwartz_10375 | Surgery_Schwartz | sarcomas, a multidisciplinary treatment approach has resulted in local control rates exceeding 90% and 5-year survival rates exceeding 70%. Most patients who die of soft tissue sarcoma die of metastatic disease, which becomes evident within 2 to 3 years of initial diagnosis in 80% of cases.Recommendations for evaluation and treatment of patients presenting with soft tissue masses are summarized in Table 36-3.Brunicardi_Ch36_p1567-p1598.indd 157401/03/19 6:38 PM 1575SOFT TISSUE SARCOMASCHAPTER 36Table 36-3Recommendations for the management of soft tissue masses1. Soft tissue tumors that are enlarging or greater than 3 cm should be evaluated with radiologic imaging (ultrasonography or computed tomography [CT]), and a tissue diagnosis should be made using core needle biopsy.2. Once a sarcoma diagnosis is established, obtain imaging (magnetic resonance imaging for extremity lesions and CT for other anatomic locations) and evaluate for metastatic disease with chest CT for | Surgery_Schwartz. sarcomas, a multidisciplinary treatment approach has resulted in local control rates exceeding 90% and 5-year survival rates exceeding 70%. Most patients who die of soft tissue sarcoma die of metastatic disease, which becomes evident within 2 to 3 years of initial diagnosis in 80% of cases.Recommendations for evaluation and treatment of patients presenting with soft tissue masses are summarized in Table 36-3.Brunicardi_Ch36_p1567-p1598.indd 157401/03/19 6:38 PM 1575SOFT TISSUE SARCOMASCHAPTER 36Table 36-3Recommendations for the management of soft tissue masses1. Soft tissue tumors that are enlarging or greater than 3 cm should be evaluated with radiologic imaging (ultrasonography or computed tomography [CT]), and a tissue diagnosis should be made using core needle biopsy.2. Once a sarcoma diagnosis is established, obtain imaging (magnetic resonance imaging for extremity lesions and CT for other anatomic locations) and evaluate for metastatic disease with chest CT for |
Surgery_Schwartz_10376 | Surgery_Schwartz | a sarcoma diagnosis is established, obtain imaging (magnetic resonance imaging for extremity lesions and CT for other anatomic locations) and evaluate for metastatic disease with chest CT for intermediateor high-grade (grade 2 or 3) or large (T2) tumors.3. A wide local excision with 1to 2-cm margins is adequate therapy for low-grade lesions and T1 tumors.4. Radiation therapy plays a critical role in the management of large (T2), intermediateor high-grade tumors.5. Patients with locally advanced high-grade sarcomas or distant metastases should be evaluated for chemotherapy.6. An aggressive surgical approach should be taken in the treatment of patients with an isolated local recurrence or resectable distant metastases.SurgeryPrimary tumors with no evidence of distant metastasis are managed with surgery alone or, when wide pathologic margins cannot be achieved because of anatomic constraints and/or the grade is high, surgery plus radiation therapy. The type of surgical resection is | Surgery_Schwartz. a sarcoma diagnosis is established, obtain imaging (magnetic resonance imaging for extremity lesions and CT for other anatomic locations) and evaluate for metastatic disease with chest CT for intermediateor high-grade (grade 2 or 3) or large (T2) tumors.3. A wide local excision with 1to 2-cm margins is adequate therapy for low-grade lesions and T1 tumors.4. Radiation therapy plays a critical role in the management of large (T2), intermediateor high-grade tumors.5. Patients with locally advanced high-grade sarcomas or distant metastases should be evaluated for chemotherapy.6. An aggressive surgical approach should be taken in the treatment of patients with an isolated local recurrence or resectable distant metastases.SurgeryPrimary tumors with no evidence of distant metastasis are managed with surgery alone or, when wide pathologic margins cannot be achieved because of anatomic constraints and/or the grade is high, surgery plus radiation therapy. The type of surgical resection is |
Surgery_Schwartz_10377 | Surgery_Schwartz | with surgery alone or, when wide pathologic margins cannot be achieved because of anatomic constraints and/or the grade is high, surgery plus radiation therapy. The type of surgical resection is determined by several factors, including tumor location, tumor size, depth of invasion, involvement of nearby structures, need for skin grafting or autogenous tissue reconstruction, and the patient’s performance status. In 1985, the National Institutes of Health developed a consensus statement recommending limb-sparing surgery for most patients with high-grade extremity sarcomas.76 However, for patients with primary or recurrent tumors that cannot be grossly resected with a limb-sparing procedure and preservation of function (<5% of patients), amputation remains the treatment of choice.Margin status after surgical resection has been shown to be an independent prognostic factor.77,78 The goal of surgical resec-tion is to achieve a complete resection because microscopically positive or grossly | Surgery_Schwartz. with surgery alone or, when wide pathologic margins cannot be achieved because of anatomic constraints and/or the grade is high, surgery plus radiation therapy. The type of surgical resection is determined by several factors, including tumor location, tumor size, depth of invasion, involvement of nearby structures, need for skin grafting or autogenous tissue reconstruction, and the patient’s performance status. In 1985, the National Institutes of Health developed a consensus statement recommending limb-sparing surgery for most patients with high-grade extremity sarcomas.76 However, for patients with primary or recurrent tumors that cannot be grossly resected with a limb-sparing procedure and preservation of function (<5% of patients), amputation remains the treatment of choice.Margin status after surgical resection has been shown to be an independent prognostic factor.77,78 The goal of surgical resec-tion is to achieve a complete resection because microscopically positive or grossly |
Surgery_Schwartz_10378 | Surgery_Schwartz | after surgical resection has been shown to be an independent prognostic factor.77,78 The goal of surgical resec-tion is to achieve a complete resection because microscopically positive or grossly positive resection margins are associated with increased risk of local recurrence and death.79 If an unex-pected positive margin is found on pathologic examination of the resection specimen, reexcision should be performed if fea-sible. In patients with a positive margin, particularly in patients with macroscopic residual disease, local control is unlikely even with the addition of postoperative radiation therapy, emphasiz-ing the importance of a well-planned initial operation.80Wide Local Excision. The preferred treatment for extrem-ity sarcomas is wide local excision that includes resection of the biopsy site. The goal of wide local excision is to remove the tumor with approximately 1 to 2 cm of surrounding nor-mal soft tissue,77 but narrower margins may be necessary to preserve uninvolved | Surgery_Schwartz. after surgical resection has been shown to be an independent prognostic factor.77,78 The goal of surgical resec-tion is to achieve a complete resection because microscopically positive or grossly positive resection margins are associated with increased risk of local recurrence and death.79 If an unex-pected positive margin is found on pathologic examination of the resection specimen, reexcision should be performed if fea-sible. In patients with a positive margin, particularly in patients with macroscopic residual disease, local control is unlikely even with the addition of postoperative radiation therapy, emphasiz-ing the importance of a well-planned initial operation.80Wide Local Excision. The preferred treatment for extrem-ity sarcomas is wide local excision that includes resection of the biopsy site. The goal of wide local excision is to remove the tumor with approximately 1 to 2 cm of surrounding nor-mal soft tissue,77 but narrower margins may be necessary to preserve uninvolved |
Surgery_Schwartz_10379 | Surgery_Schwartz | the biopsy site. The goal of wide local excision is to remove the tumor with approximately 1 to 2 cm of surrounding nor-mal soft tissue,77 but narrower margins may be necessary to preserve uninvolved critical neurovascular structures and may be adequate for patients undergoing radiation therapy.81 Dis-section should proceed through grossly normal tissue planes not abutting the tumor. Soft tissue sarcomas are generally sur-rounded by a zone of compressed reactive tissue that forms a pseudocapsule; this pseudocapsule should not be used to guide resection (enucleation) as microscopic disease exists within this reactive zone. If the tumor is adjacent to or displacing major neurovascular structures, these do not need to be resected, but the adventitia or perineurium should be removed.1 For some massive tumors of the extremities, wide local excision entails a radical or complete anatomic compartment resection. Surgical clips should be placed to delineate the extent of the resection bed for | Surgery_Schwartz. the biopsy site. The goal of wide local excision is to remove the tumor with approximately 1 to 2 cm of surrounding nor-mal soft tissue,77 but narrower margins may be necessary to preserve uninvolved critical neurovascular structures and may be adequate for patients undergoing radiation therapy.81 Dis-section should proceed through grossly normal tissue planes not abutting the tumor. Soft tissue sarcomas are generally sur-rounded by a zone of compressed reactive tissue that forms a pseudocapsule; this pseudocapsule should not be used to guide resection (enucleation) as microscopic disease exists within this reactive zone. If the tumor is adjacent to or displacing major neurovascular structures, these do not need to be resected, but the adventitia or perineurium should be removed.1 For some massive tumors of the extremities, wide local excision entails a radical or complete anatomic compartment resection. Surgical clips should be placed to delineate the extent of the resection bed for |
Surgery_Schwartz_10380 | Surgery_Schwartz | massive tumors of the extremities, wide local excision entails a radical or complete anatomic compartment resection. Surgical clips should be placed to delineate the extent of the resection bed for patients likely to require postoperative radiation therapy.Recent reports demonstrate encouraging results follow-ing radical en bloc resection with vascular reconstruction in the lower extremities.82,83 While en bloc resection with vascular reconstruction has been associated with increased rates of post-operative complications, reported local recurrence and 5-year survival rates are similar to those for patients not requiring ves-sel resection.84,85 Similarly, studies have shown acceptable func-tional outcomes with resection of the sciatic, tibial, and peroneal nerves with appropriate reconstruction and rehabilitation.86Bone invasion from extremity soft tissue sarcoma, which can generally be identified using high-quality cross-sectional imaging such as MRI, has been estimated to occur in | Surgery_Schwartz. massive tumors of the extremities, wide local excision entails a radical or complete anatomic compartment resection. Surgical clips should be placed to delineate the extent of the resection bed for patients likely to require postoperative radiation therapy.Recent reports demonstrate encouraging results follow-ing radical en bloc resection with vascular reconstruction in the lower extremities.82,83 While en bloc resection with vascular reconstruction has been associated with increased rates of post-operative complications, reported local recurrence and 5-year survival rates are similar to those for patients not requiring ves-sel resection.84,85 Similarly, studies have shown acceptable func-tional outcomes with resection of the sciatic, tibial, and peroneal nerves with appropriate reconstruction and rehabilitation.86Bone invasion from extremity soft tissue sarcoma, which can generally be identified using high-quality cross-sectional imaging such as MRI, has been estimated to occur in |
Surgery_Schwartz_10381 | Surgery_Schwartz | and rehabilitation.86Bone invasion from extremity soft tissue sarcoma, which can generally be identified using high-quality cross-sectional imaging such as MRI, has been estimated to occur in about 5% of patients and is associated with reduced overall survival.87 In cases of bone invasion, bone resection is required to obtain an adequate surgical margin and to achieve local control. Although tumor resection and repair of skeletal defects are possible, the likelihood of postoperative complications may be increased, and functional outcomes may be less favorable. Lin and colleagues88 recently analyzed 55 patients with soft tissue sarcomas abutting bone and reported that in the absence of frank cortical bone pen-etration, periosteum was an adequate surgical margin in patients treated with wide local excision and radiation.Soft tissue sarcomas arising in the distal extremities, particularly the hands and feet, present unique technical chal-lenges. While distal-extremity tumors are often | Surgery_Schwartz. and rehabilitation.86Bone invasion from extremity soft tissue sarcoma, which can generally be identified using high-quality cross-sectional imaging such as MRI, has been estimated to occur in about 5% of patients and is associated with reduced overall survival.87 In cases of bone invasion, bone resection is required to obtain an adequate surgical margin and to achieve local control. Although tumor resection and repair of skeletal defects are possible, the likelihood of postoperative complications may be increased, and functional outcomes may be less favorable. Lin and colleagues88 recently analyzed 55 patients with soft tissue sarcomas abutting bone and reported that in the absence of frank cortical bone pen-etration, periosteum was an adequate surgical margin in patients treated with wide local excision and radiation.Soft tissue sarcomas arising in the distal extremities, particularly the hands and feet, present unique technical chal-lenges. While distal-extremity tumors are often |
Surgery_Schwartz_10382 | Surgery_Schwartz | local excision and radiation.Soft tissue sarcomas arising in the distal extremities, particularly the hands and feet, present unique technical chal-lenges. While distal-extremity tumors are often detected at a smaller size (<5 cm) than proximal-extremity tumors, resec-tion and reconstruction techniques are often more complex for distal-extremity tumors, and preoperative planning is criti-cal to obtain favorable functional outcomes. Identifying the proximity of the tumor to underlying critical structures (e.g., bone, tendon, or neurovascular structures) using MRI is essen-tial for surgical planning. In a reported series of patients with sarcomas of the hands or feet treated with limited surgery only, 32% of patients had local recurrences.89 Preservation of function and acceptable recurrence rates with limited surgery and adjuvant radiation therapy for soft tissue sarcomas of the distal extremities have been reported.90 For locally advanced tumors, repair of bone defects, vascular | Surgery_Schwartz. local excision and radiation.Soft tissue sarcomas arising in the distal extremities, particularly the hands and feet, present unique technical chal-lenges. While distal-extremity tumors are often detected at a smaller size (<5 cm) than proximal-extremity tumors, resec-tion and reconstruction techniques are often more complex for distal-extremity tumors, and preoperative planning is criti-cal to obtain favorable functional outcomes. Identifying the proximity of the tumor to underlying critical structures (e.g., bone, tendon, or neurovascular structures) using MRI is essen-tial for surgical planning. In a reported series of patients with sarcomas of the hands or feet treated with limited surgery only, 32% of patients had local recurrences.89 Preservation of function and acceptable recurrence rates with limited surgery and adjuvant radiation therapy for soft tissue sarcomas of the distal extremities have been reported.90 For locally advanced tumors, repair of bone defects, vascular |
Surgery_Schwartz_10383 | Surgery_Schwartz | rates with limited surgery and adjuvant radiation therapy for soft tissue sarcomas of the distal extremities have been reported.90 For locally advanced tumors, repair of bone defects, vascular reconstruction, tendon transfers, and soft tissue reconstruction using regional or free flaps have resulted in good functional outcomes.91 Amputation remains a reasonable option for patients with soft tissue sar-comas of the distal extremities when acceptable oncologic or functional outcomes cannot be achieved using available limb salvage techniques.In an interesting study conducted in Ontario and Quebec, investigators found patients expecting a difficult recovery and patients with uncertain expectations had worse functional out-comes than patients anticipating an easy recovery, indicating that preoperative education including consultation with reha-bilitation services may optimize outcomes.92 Furthermore, all patients undergoing resection of extremity sarcomas should undergo physical therapy | Surgery_Schwartz. rates with limited surgery and adjuvant radiation therapy for soft tissue sarcomas of the distal extremities have been reported.90 For locally advanced tumors, repair of bone defects, vascular reconstruction, tendon transfers, and soft tissue reconstruction using regional or free flaps have resulted in good functional outcomes.91 Amputation remains a reasonable option for patients with soft tissue sar-comas of the distal extremities when acceptable oncologic or functional outcomes cannot be achieved using available limb salvage techniques.In an interesting study conducted in Ontario and Quebec, investigators found patients expecting a difficult recovery and patients with uncertain expectations had worse functional out-comes than patients anticipating an easy recovery, indicating that preoperative education including consultation with reha-bilitation services may optimize outcomes.92 Furthermore, all patients undergoing resection of extremity sarcomas should undergo physical therapy |
Surgery_Schwartz_10384 | Surgery_Schwartz | education including consultation with reha-bilitation services may optimize outcomes.92 Furthermore, all patients undergoing resection of extremity sarcomas should undergo physical therapy beginning immediately after surgery and continuing until maximum function is achieved.1Brunicardi_Ch36_p1567-p1598.indd 157501/03/19 6:38 PM 1576SPECIFIC CONSIDERATIONSPART IILocoregional Lymphadenectomy. Several studies have reported improved survival for patients with isolated regional lymph node metastases treated with radical lymphadenectomy.28,63-65 Patients with clinically or radiologically suspicious regional nodes should have metastases confirmed by biopsy before radi-cal lymphadenectomy. An ultrasound-guided fine-needle aspi-ration or core biopsy of lymph nodes in selected patients with suspicious clinical or radiologic findings. The utility of sentinel lymph node biopsy has remained controversial despite the rec-ognition that several histologic subtypes of high-grade sarcoma are known | Surgery_Schwartz. education including consultation with reha-bilitation services may optimize outcomes.92 Furthermore, all patients undergoing resection of extremity sarcomas should undergo physical therapy beginning immediately after surgery and continuing until maximum function is achieved.1Brunicardi_Ch36_p1567-p1598.indd 157501/03/19 6:38 PM 1576SPECIFIC CONSIDERATIONSPART IILocoregional Lymphadenectomy. Several studies have reported improved survival for patients with isolated regional lymph node metastases treated with radical lymphadenectomy.28,63-65 Patients with clinically or radiologically suspicious regional nodes should have metastases confirmed by biopsy before radi-cal lymphadenectomy. An ultrasound-guided fine-needle aspi-ration or core biopsy of lymph nodes in selected patients with suspicious clinical or radiologic findings. The utility of sentinel lymph node biopsy has remained controversial despite the rec-ognition that several histologic subtypes of high-grade sarcoma are known |
Surgery_Schwartz_10385 | Surgery_Schwartz | clinical or radiologic findings. The utility of sentinel lymph node biopsy has remained controversial despite the rec-ognition that several histologic subtypes of high-grade sarcoma are known to have a propensity for lymph node metastasis. However, there have been no prospective studies of the sen-sitivity and specificity of sentinel lymph node biopsy for such tumors, and as such, sentinel node biopsy for sarcoma should be performed in either highly selected patients or in the setting of a clinical trial.Amputation. Amputation is the treatment of choice for the 5% of patients with primary or recurrent extremity tumors whose tumors cannot be grossly resected with limb-sparing proce-dures and preservation of function. Historically, local excision of large, high-grade soft tissue sarcomas resulted in local fail-ure rates of 50% to 70%, even when a margin of normal tissue around the tumor was excised; consequently, radical resection or amputation was recommended. Today, however, the | Surgery_Schwartz. clinical or radiologic findings. The utility of sentinel lymph node biopsy has remained controversial despite the rec-ognition that several histologic subtypes of high-grade sarcoma are known to have a propensity for lymph node metastasis. However, there have been no prospective studies of the sen-sitivity and specificity of sentinel lymph node biopsy for such tumors, and as such, sentinel node biopsy for sarcoma should be performed in either highly selected patients or in the setting of a clinical trial.Amputation. Amputation is the treatment of choice for the 5% of patients with primary or recurrent extremity tumors whose tumors cannot be grossly resected with limb-sparing proce-dures and preservation of function. Historically, local excision of large, high-grade soft tissue sarcomas resulted in local fail-ure rates of 50% to 70%, even when a margin of normal tissue around the tumor was excised; consequently, radical resection or amputation was recommended. Today, however, the |
Surgery_Schwartz_10386 | Surgery_Schwartz | resulted in local fail-ure rates of 50% to 70%, even when a margin of normal tissue around the tumor was excised; consequently, radical resection or amputation was recommended. Today, however, the addition of radiation therapy to less radical surgical resection has made limb salvage possible in most cases.A comparison of amputation versus limb-sparing sur-gery followed by adjuvant radiation therapy performed by the National Cancer Institute between 1975 and 1981 demonstrated no significant difference between the two groups in local recur-rence or overall survival rate.93 Potter and colleagues50 later reviewed the entire National Cancer Institute experience with 123 patients treated with conservative surgery plus radiation therapy and 83 treated with amputation. The local recurrence rate was significantly higher in the surgery and adjuvant radia-tion therapy group: 8% versus 0% in the amputation group. However, survival rates did not differ between the groups. Several large | Surgery_Schwartz. resulted in local fail-ure rates of 50% to 70%, even when a margin of normal tissue around the tumor was excised; consequently, radical resection or amputation was recommended. Today, however, the addition of radiation therapy to less radical surgical resection has made limb salvage possible in most cases.A comparison of amputation versus limb-sparing sur-gery followed by adjuvant radiation therapy performed by the National Cancer Institute between 1975 and 1981 demonstrated no significant difference between the two groups in local recur-rence or overall survival rate.93 Potter and colleagues50 later reviewed the entire National Cancer Institute experience with 123 patients treated with conservative surgery plus radiation therapy and 83 treated with amputation. The local recurrence rate was significantly higher in the surgery and adjuvant radia-tion therapy group: 8% versus 0% in the amputation group. However, survival rates did not differ between the groups. Several large |
Surgery_Schwartz_10387 | Surgery_Schwartz | rate was significantly higher in the surgery and adjuvant radia-tion therapy group: 8% versus 0% in the amputation group. However, survival rates did not differ between the groups. Several large single-institution studies have since also reported favorable local control rates with conservative resection plus radiation therapy.94-96Isolated Regional Perfusion. Isolated regional perfusion is a limb-sparing technique in which a soft tissue sarcoma is perfused with high concentrations of tumor necrosis factor-alpha (TNF-α) and melphalan under hyperthermic conditions. The use of TNF-α is not approved by the U.S. Food and Drug Administration (FDA) and is used only in European countries. The technique is generally used for locally advanced, multifo-cal, or locally recurrent disease; it has also served as a pallia-tive treatment to achieve local control for patients with distant metastases.Limb perfusion requires isolating the main artery and vein of the perfused limb from the systemic | Surgery_Schwartz. rate was significantly higher in the surgery and adjuvant radia-tion therapy group: 8% versus 0% in the amputation group. However, survival rates did not differ between the groups. Several large single-institution studies have since also reported favorable local control rates with conservative resection plus radiation therapy.94-96Isolated Regional Perfusion. Isolated regional perfusion is a limb-sparing technique in which a soft tissue sarcoma is perfused with high concentrations of tumor necrosis factor-alpha (TNF-α) and melphalan under hyperthermic conditions. The use of TNF-α is not approved by the U.S. Food and Drug Administration (FDA) and is used only in European countries. The technique is generally used for locally advanced, multifo-cal, or locally recurrent disease; it has also served as a pallia-tive treatment to achieve local control for patients with distant metastases.Limb perfusion requires isolating the main artery and vein of the perfused limb from the systemic |
Surgery_Schwartz_10388 | Surgery_Schwartz | also served as a pallia-tive treatment to achieve local control for patients with distant metastases.Limb perfusion requires isolating the main artery and vein of the perfused limb from the systemic circulation. The ana-tomic approach is determined by tumor site: external iliac ves-sels are used for thigh tumors, femoral or popliteal vessels for calf tumors, and axillary vessels for upper extremity tumors. The vessels are dissected, and all collateral vessels are ligated. The main artery and vein are then cannulated and connected to a pump oxygenator similar to that used in cardiopulmonary bypass. Either a tourniquet or an Esmarch band is applied to the limb to achieve complete vascular isolation. Chemotherapeutic agents are then added to the perfusion circuit and circulated for 90 minutes. Systemic leakage from the perfused limb is moni-tored continuously with 99Tc-radiolabeled human serum albumin injected into the perfusate, and radioactivity above the precor-dial area is recorded | Surgery_Schwartz. also served as a pallia-tive treatment to achieve local control for patients with distant metastases.Limb perfusion requires isolating the main artery and vein of the perfused limb from the systemic circulation. The ana-tomic approach is determined by tumor site: external iliac ves-sels are used for thigh tumors, femoral or popliteal vessels for calf tumors, and axillary vessels for upper extremity tumors. The vessels are dissected, and all collateral vessels are ligated. The main artery and vein are then cannulated and connected to a pump oxygenator similar to that used in cardiopulmonary bypass. Either a tourniquet or an Esmarch band is applied to the limb to achieve complete vascular isolation. Chemotherapeutic agents are then added to the perfusion circuit and circulated for 90 minutes. Systemic leakage from the perfused limb is moni-tored continuously with 99Tc-radiolabeled human serum albumin injected into the perfusate, and radioactivity above the precor-dial area is recorded |
Surgery_Schwartz_10389 | Surgery_Schwartz | Systemic leakage from the perfused limb is moni-tored continuously with 99Tc-radiolabeled human serum albumin injected into the perfusate, and radioactivity above the precor-dial area is recorded with a Geiger counter. During the entire procedure, hyperthermia of the perfused limb is maintained by external heating and by warming the perfusate to 40°C. At the end of the procedure, the limb is washed out, the cannulas are extracted, and the blood vessels are repaired.Despite the 40-year history of using isolated limb per-fusion to treat extremity sarcomas, many questions about this technique remain to be answered. The optimal chemotherapeu-tic agent in the perfusion circuit, the benefits of hyperthermia, and the effectiveness of hyperthermic perfusion as neoadjuvant or adjuvant treatment remain to be elucidated. Studies published to date have involved heterogeneous patient groups and various chemotherapeutic agents. Despite these limitations, response rates from 18% to 80% and overall | Surgery_Schwartz. Systemic leakage from the perfused limb is moni-tored continuously with 99Tc-radiolabeled human serum albumin injected into the perfusate, and radioactivity above the precor-dial area is recorded with a Geiger counter. During the entire procedure, hyperthermia of the perfused limb is maintained by external heating and by warming the perfusate to 40°C. At the end of the procedure, the limb is washed out, the cannulas are extracted, and the blood vessels are repaired.Despite the 40-year history of using isolated limb per-fusion to treat extremity sarcomas, many questions about this technique remain to be answered. The optimal chemotherapeu-tic agent in the perfusion circuit, the benefits of hyperthermia, and the effectiveness of hyperthermic perfusion as neoadjuvant or adjuvant treatment remain to be elucidated. Studies published to date have involved heterogeneous patient groups and various chemotherapeutic agents. Despite these limitations, response rates from 18% to 80% and overall |
Surgery_Schwartz_10390 | Surgery_Schwartz | to be elucidated. Studies published to date have involved heterogeneous patient groups and various chemotherapeutic agents. Despite these limitations, response rates from 18% to 80% and overall 5-year survival rates from 50% to 70% have been reported.97-101 However, survival out-comes following isolated limb perfusion have not yet been directly compared with survival outcomes after more conven-tional treatment approaches.In the initial report of isolated regional perfusion for extremity sarcomas, published in 1974, McBride reported results in 79 patients with extremity sarcomas who had been treated with isolated limb perfusion during the previous 14 years.97 All patients received melphalan and dactinomycin. The overall 5-year survival rate was 57%, and only 13 patients had subsequent amputation for recurrent disease. Over the next 20 years, isolated perfusion for treatment of extremity sarcoma fell out of favor for several reasons. Most notably, improved survival and decreased local | Surgery_Schwartz. to be elucidated. Studies published to date have involved heterogeneous patient groups and various chemotherapeutic agents. Despite these limitations, response rates from 18% to 80% and overall 5-year survival rates from 50% to 70% have been reported.97-101 However, survival out-comes following isolated limb perfusion have not yet been directly compared with survival outcomes after more conven-tional treatment approaches.In the initial report of isolated regional perfusion for extremity sarcomas, published in 1974, McBride reported results in 79 patients with extremity sarcomas who had been treated with isolated limb perfusion during the previous 14 years.97 All patients received melphalan and dactinomycin. The overall 5-year survival rate was 57%, and only 13 patients had subsequent amputation for recurrent disease. Over the next 20 years, isolated perfusion for treatment of extremity sarcoma fell out of favor for several reasons. Most notably, improved survival and decreased local |
Surgery_Schwartz_10391 | Surgery_Schwartz | for recurrent disease. Over the next 20 years, isolated perfusion for treatment of extremity sarcoma fell out of favor for several reasons. Most notably, improved survival and decreased local recurrence rates could be obtained with less radical therapy, including conservative surgical exci-sion combined with radiation to allow limb sparing in patients who were previously thought to require amputation.A 1992 report by Lienard and colleagues101 renewed interest in isolated limb perfusion for extremity tumors. Those investigators reported a 100% response rate among patients with extremity melanomas and sarcomas treated with high-dose recombinant TNF-α plus interferon-γ and melphalan in an iso-lated perfusion circuit. This report led to larger studies geared specifically to patients with sarcoma. The largest of these stud-ies, the European Multicenter Study, was reported by Egger-mont and colleagues in 1996.99 In that study of 186 patients, the overall tumor response rate was 82%, and the | Surgery_Schwartz. for recurrent disease. Over the next 20 years, isolated perfusion for treatment of extremity sarcoma fell out of favor for several reasons. Most notably, improved survival and decreased local recurrence rates could be obtained with less radical therapy, including conservative surgical exci-sion combined with radiation to allow limb sparing in patients who were previously thought to require amputation.A 1992 report by Lienard and colleagues101 renewed interest in isolated limb perfusion for extremity tumors. Those investigators reported a 100% response rate among patients with extremity melanomas and sarcomas treated with high-dose recombinant TNF-α plus interferon-γ and melphalan in an iso-lated perfusion circuit. This report led to larger studies geared specifically to patients with sarcoma. The largest of these stud-ies, the European Multicenter Study, was reported by Egger-mont and colleagues in 1996.99 In that study of 186 patients, the overall tumor response rate was 82%, and the |
Surgery_Schwartz_10392 | Surgery_Schwartz | The largest of these stud-ies, the European Multicenter Study, was reported by Egger-mont and colleagues in 1996.99 In that study of 186 patients, the overall tumor response rate was 82%, and the clinical and pathologic complete response rate was 29%. Although all of the study participants were reported to initially be candidates for amputation, the rate of limb salvage following isolated limb perfusion was 82%.99 Subsequent studies have shown high local response and limb salvage rates and acceptable local and sys-temic toxic effects.102However, results in the United States have been inferior to those reported in Europe. In a study by Fraker and colleagues, the complete response rate was 26%, and an additional 30% of patients had a partial response. Fourteen patients (32%) under-went amputation for progressive tumors, while the remaining 30 patients (68%) were able to undergo limb-sparing surgery after isolated limb perfusion.100 The inferior results in the U.S.-based studies are | Surgery_Schwartz. The largest of these stud-ies, the European Multicenter Study, was reported by Egger-mont and colleagues in 1996.99 In that study of 186 patients, the overall tumor response rate was 82%, and the clinical and pathologic complete response rate was 29%. Although all of the study participants were reported to initially be candidates for amputation, the rate of limb salvage following isolated limb perfusion was 82%.99 Subsequent studies have shown high local response and limb salvage rates and acceptable local and sys-temic toxic effects.102However, results in the United States have been inferior to those reported in Europe. In a study by Fraker and colleagues, the complete response rate was 26%, and an additional 30% of patients had a partial response. Fourteen patients (32%) under-went amputation for progressive tumors, while the remaining 30 patients (68%) were able to undergo limb-sparing surgery after isolated limb perfusion.100 The inferior results in the U.S.-based studies are |
Surgery_Schwartz_10393 | Surgery_Schwartz | amputation for progressive tumors, while the remaining 30 patients (68%) were able to undergo limb-sparing surgery after isolated limb perfusion.100 The inferior results in the U.S.-based studies are thought to be due to patient selection biases and the degree of treatment before limb perfusion.While isolated limb perfusion for extremity sarcoma has fallen out of favor, recent reports of isolated limb infusion from the H. Lee Moffitt Cancer Center have shown promising Brunicardi_Ch36_p1567-p1598.indd 157601/03/19 6:38 PM 1577SOFT TISSUE SARCOMASCHAPTER 36results.103 Patients with extremity sarcoma and who are con-sidered for amputation are offered the option of isolated limb infusion with high-dose melphalan and actinomycin-D on pro-tocol. Isolated limb infusion is a less invasive technique that can be repeated. Percutaneous cannulas are placed prior to infu-sion, the extremity is isolated similar to limb perfusion with an Esmarch band or tourniquet, and the perfusion is | Surgery_Schwartz. amputation for progressive tumors, while the remaining 30 patients (68%) were able to undergo limb-sparing surgery after isolated limb perfusion.100 The inferior results in the U.S.-based studies are thought to be due to patient selection biases and the degree of treatment before limb perfusion.While isolated limb perfusion for extremity sarcoma has fallen out of favor, recent reports of isolated limb infusion from the H. Lee Moffitt Cancer Center have shown promising Brunicardi_Ch36_p1567-p1598.indd 157601/03/19 6:38 PM 1577SOFT TISSUE SARCOMASCHAPTER 36results.103 Patients with extremity sarcoma and who are con-sidered for amputation are offered the option of isolated limb infusion with high-dose melphalan and actinomycin-D on pro-tocol. Isolated limb infusion is a less invasive technique that can be repeated. Percutaneous cannulas are placed prior to infu-sion, the extremity is isolated similar to limb perfusion with an Esmarch band or tourniquet, and the perfusion is |
Surgery_Schwartz_10394 | Surgery_Schwartz | technique that can be repeated. Percutaneous cannulas are placed prior to infu-sion, the extremity is isolated similar to limb perfusion with an Esmarch band or tourniquet, and the perfusion is normothermic and acidotic. Results from a multicenter retrospective study have demonstrated an overall response rate of 58%, and after a median follow-up of 21 months, there was an overall limb sal-vage rate of 78%. The benefits over limb perfusion remain the ability to repeat the technique in patients with disease response and less operative morbidity and risk of vascular injury from open surgery and cannulation of the iliac vessels. Although to date the technique has been well established for patients with locally advanced extremity disease for melanoma, its applica-tion for advanced, locally recurrent extremity sarcoma deserves further study.Radiation TherapyRadiation therapy is part of the standard treatment for high-grade extremity and trunk wall soft tissue sarcomas either in the preor | Surgery_Schwartz. technique that can be repeated. Percutaneous cannulas are placed prior to infu-sion, the extremity is isolated similar to limb perfusion with an Esmarch band or tourniquet, and the perfusion is normothermic and acidotic. Results from a multicenter retrospective study have demonstrated an overall response rate of 58%, and after a median follow-up of 21 months, there was an overall limb sal-vage rate of 78%. The benefits over limb perfusion remain the ability to repeat the technique in patients with disease response and less operative morbidity and risk of vascular injury from open surgery and cannulation of the iliac vessels. Although to date the technique has been well established for patients with locally advanced extremity disease for melanoma, its applica-tion for advanced, locally recurrent extremity sarcoma deserves further study.Radiation TherapyRadiation therapy is part of the standard treatment for high-grade extremity and trunk wall soft tissue sarcomas either in the preor |
Surgery_Schwartz_10395 | Surgery_Schwartz | extremity sarcoma deserves further study.Radiation TherapyRadiation therapy is part of the standard treatment for high-grade extremity and trunk wall soft tissue sarcomas either in the preor postoperative setting. Patients with low-grade tumors or small, superficial high-grade tumors that have been resected with adequate margins may safely avoid radiation therapy.The evidence supporting adjuvant radiation therapy for patients eligible for conservative surgical resection comes from two randomized trials104,105 and three large single-institution reports.106-108 In a randomized trial by the National Cancer Institute, 91 patients with high-grade extremity tumors were treated with limb-sparing surgery followed by chemotherapy alone or radiation therapy plus chemotherapy. The 10-year local control rate was 98% for patients receiving radiation therapy compared with 70% for those not receiving radiation therapy (P = .0001).104 Similarly, in a randomized trial from Memorial Sloan-Kettering | Surgery_Schwartz. extremity sarcoma deserves further study.Radiation TherapyRadiation therapy is part of the standard treatment for high-grade extremity and trunk wall soft tissue sarcomas either in the preor postoperative setting. Patients with low-grade tumors or small, superficial high-grade tumors that have been resected with adequate margins may safely avoid radiation therapy.The evidence supporting adjuvant radiation therapy for patients eligible for conservative surgical resection comes from two randomized trials104,105 and three large single-institution reports.106-108 In a randomized trial by the National Cancer Institute, 91 patients with high-grade extremity tumors were treated with limb-sparing surgery followed by chemotherapy alone or radiation therapy plus chemotherapy. The 10-year local control rate was 98% for patients receiving radiation therapy compared with 70% for those not receiving radiation therapy (P = .0001).104 Similarly, in a randomized trial from Memorial Sloan-Kettering |
Surgery_Schwartz_10396 | Surgery_Schwartz | rate was 98% for patients receiving radiation therapy compared with 70% for those not receiving radiation therapy (P = .0001).104 Similarly, in a randomized trial from Memorial Sloan-Kettering Cancer Center, 164 patients underwent conservative surgery followed by observation or brachytherapy. For patients with high-grade tumors, the 5-year local control rate was 66% in the observation group and 89% in the brachytherapy group (P = .003).105 For patients with low-grade tumors, no significant difference was observed between treatment groups.109Until recently, the standard treatment guidelines required radiation therapy after surgery for all patients with intermediate or high-grade tumors of any size. However, small tumors (≤5 cm) have not generally been associated with local recurrence, and radiation therapy for such tumors may not be necessary.105 In a series of 174 patients reported by Geer and colleagues, post-operative radiation therapy did not improve 5-year local recur-rence or | Surgery_Schwartz. rate was 98% for patients receiving radiation therapy compared with 70% for those not receiving radiation therapy (P = .0001).104 Similarly, in a randomized trial from Memorial Sloan-Kettering Cancer Center, 164 patients underwent conservative surgery followed by observation or brachytherapy. For patients with high-grade tumors, the 5-year local control rate was 66% in the observation group and 89% in the brachytherapy group (P = .003).105 For patients with low-grade tumors, no significant difference was observed between treatment groups.109Until recently, the standard treatment guidelines required radiation therapy after surgery for all patients with intermediate or high-grade tumors of any size. However, small tumors (≤5 cm) have not generally been associated with local recurrence, and radiation therapy for such tumors may not be necessary.105 In a series of 174 patients reported by Geer and colleagues, post-operative radiation therapy did not improve 5-year local recur-rence or |
Surgery_Schwartz_10397 | Surgery_Schwartz | radiation therapy for such tumors may not be necessary.105 In a series of 174 patients reported by Geer and colleagues, post-operative radiation therapy did not improve 5-year local recur-rence or overall survival rates for patients with small soft tissue sarcomas.110 Karakousis and colleagues reported a 5-year local recurrence rate of 6% for 80 patients with extremity sarcomas treated with wide local excision and observation, a rate similar to that for the 64 patients who underwent resection with nar-rower surgical margins and postoperative radiation therapy.111The optimal mode of radiation therapy (external-beam radiation therapy, brachytherapy, or intensity-modulated radia-tion therapy [IMRT]) and timing of radiation therapy (preopera-tive, intraoperative, or postoperative) have yet to be defined. External-beam radiation therapy can be delivered using pho-tons or particle beams (electrons, protons, pions, or neutrons). Conventional fractionation is usually 1.8 to 2 Gy per day. CT | Surgery_Schwartz. radiation therapy for such tumors may not be necessary.105 In a series of 174 patients reported by Geer and colleagues, post-operative radiation therapy did not improve 5-year local recur-rence or overall survival rates for patients with small soft tissue sarcomas.110 Karakousis and colleagues reported a 5-year local recurrence rate of 6% for 80 patients with extremity sarcomas treated with wide local excision and observation, a rate similar to that for the 64 patients who underwent resection with nar-rower surgical margins and postoperative radiation therapy.111The optimal mode of radiation therapy (external-beam radiation therapy, brachytherapy, or intensity-modulated radia-tion therapy [IMRT]) and timing of radiation therapy (preopera-tive, intraoperative, or postoperative) have yet to be defined. External-beam radiation therapy can be delivered using pho-tons or particle beams (electrons, protons, pions, or neutrons). Conventional fractionation is usually 1.8 to 2 Gy per day. CT |
Surgery_Schwartz_10398 | Surgery_Schwartz | be defined. External-beam radiation therapy can be delivered using pho-tons or particle beams (electrons, protons, pions, or neutrons). Conventional fractionation is usually 1.8 to 2 Gy per day. CT is an integral part of radiation therapy, used to define the gross tumor volume and to estimate the margin of tissue at risk for microscopic tumor involvement. The optimal radiation margin is not well defined: a margin of 5 to 7 cm is standard, but some centers advocate wider margins for tumors larger than 15 cm. At most institutions, the typical preoperative dose is 50 Gy given in 25 fractions, and resection is performed 4 to 8 weeks after completion of radiation therapy to allow acute radiation changes to subside. Postoperative radiation therapy planning is based on tumor site, tumor grade, surgical margins, and institutional preferences. The entire surgical scar and drain sites should be included in the field so that a near-full dose can be administered to the superficial skin. Metallic | Surgery_Schwartz. be defined. External-beam radiation therapy can be delivered using pho-tons or particle beams (electrons, protons, pions, or neutrons). Conventional fractionation is usually 1.8 to 2 Gy per day. CT is an integral part of radiation therapy, used to define the gross tumor volume and to estimate the margin of tissue at risk for microscopic tumor involvement. The optimal radiation margin is not well defined: a margin of 5 to 7 cm is standard, but some centers advocate wider margins for tumors larger than 15 cm. At most institutions, the typical preoperative dose is 50 Gy given in 25 fractions, and resection is performed 4 to 8 weeks after completion of radiation therapy to allow acute radiation changes to subside. Postoperative radiation therapy planning is based on tumor site, tumor grade, surgical margins, and institutional preferences. The entire surgical scar and drain sites should be included in the field so that a near-full dose can be administered to the superficial skin. Metallic |
Surgery_Schwartz_10399 | Surgery_Schwartz | margins, and institutional preferences. The entire surgical scar and drain sites should be included in the field so that a near-full dose can be administered to the superficial skin. Metallic clips placed in the tumor bed during surgery can help define the limits of the resection and aid in radiation therapy planning. Doses of 60 to 70 Gy are usually necessary for postoperative treatment.No consensus exists on the optimal sequence of radia-tion therapy and surgery. The available data come largely from single-institution, nonrandomized studies. Proponents of pre-operative radiation therapy note that multidisciplinary planning with radiation oncologists, medical oncologists, and surgeons is easier early in the course of therapy. In addition, for some radiosensitive histologic subtypes, such as myxoid liposarcoma, preoperative radiation therapy may shrink the tumor, facilitat-ing resection with negative margins. Furthermore, a tissue bed undisturbed by resection has better tissue | Surgery_Schwartz. margins, and institutional preferences. The entire surgical scar and drain sites should be included in the field so that a near-full dose can be administered to the superficial skin. Metallic clips placed in the tumor bed during surgery can help define the limits of the resection and aid in radiation therapy planning. Doses of 60 to 70 Gy are usually necessary for postoperative treatment.No consensus exists on the optimal sequence of radia-tion therapy and surgery. The available data come largely from single-institution, nonrandomized studies. Proponents of pre-operative radiation therapy note that multidisciplinary planning with radiation oncologists, medical oncologists, and surgeons is easier early in the course of therapy. In addition, for some radiosensitive histologic subtypes, such as myxoid liposarcoma, preoperative radiation therapy may shrink the tumor, facilitat-ing resection with negative margins. Furthermore, a tissue bed undisturbed by resection has better tissue |
Surgery_Schwartz_10400 | Surgery_Schwartz | such as myxoid liposarcoma, preoperative radiation therapy may shrink the tumor, facilitat-ing resection with negative margins. Furthermore, a tissue bed undisturbed by resection has better tissue oxygenation and can be successfully treated with lower doses of radiation. In addi-tion, Nielsen and colleagues112 demonstrated that preoperative radiation fields are smaller than postoperative radiation fields and that the average number of joints included in the field is lower with preoperative than postoperative radiation therapy, which may result in improved functional outcome. Critics of preoperative radiation therapy cite the difficulty of pathologic assessment of margins and the increased rate of postoperative wound complications.113 However, reconstructive surgical tech-niques with advanced tissue transfer procedures are being used more often in these high-risk wounds and reportedly result in better outcomes. The higher doses generally required for post-operative radiation therapy | Surgery_Schwartz. such as myxoid liposarcoma, preoperative radiation therapy may shrink the tumor, facilitat-ing resection with negative margins. Furthermore, a tissue bed undisturbed by resection has better tissue oxygenation and can be successfully treated with lower doses of radiation. In addi-tion, Nielsen and colleagues112 demonstrated that preoperative radiation fields are smaller than postoperative radiation fields and that the average number of joints included in the field is lower with preoperative than postoperative radiation therapy, which may result in improved functional outcome. Critics of preoperative radiation therapy cite the difficulty of pathologic assessment of margins and the increased rate of postoperative wound complications.113 However, reconstructive surgical tech-niques with advanced tissue transfer procedures are being used more often in these high-risk wounds and reportedly result in better outcomes. The higher doses generally required for post-operative radiation therapy |
Surgery_Schwartz_10401 | Surgery_Schwartz | tissue transfer procedures are being used more often in these high-risk wounds and reportedly result in better outcomes. The higher doses generally required for post-operative radiation therapy have also been shown to be associ-ated with greater long-term functional impairment.The only randomized comparison of preoperative and postoperative radiation therapy to date was performed by the National Cancer Institute of Canada Clinical Trials–Canadian Sarcoma Group.114 This trial was designed to examine compli-cations and functional outcome. The 190 patients enrolled from October 1994 to December 1997 were randomized to preopera-tive radiation therapy (50 Gy) or postoperative radiation therapy (66 Gy). With a median follow-up time of 3.3 years, the recur-rence and progression-free survival rates were similar in the two groups. The incidence of wound complications was higher in the preoperative group versus the postoperative group (35% vs. 17%), and the incidence of wound complications was | Surgery_Schwartz. tissue transfer procedures are being used more often in these high-risk wounds and reportedly result in better outcomes. The higher doses generally required for post-operative radiation therapy have also been shown to be associ-ated with greater long-term functional impairment.The only randomized comparison of preoperative and postoperative radiation therapy to date was performed by the National Cancer Institute of Canada Clinical Trials–Canadian Sarcoma Group.114 This trial was designed to examine compli-cations and functional outcome. The 190 patients enrolled from October 1994 to December 1997 were randomized to preopera-tive radiation therapy (50 Gy) or postoperative radiation therapy (66 Gy). With a median follow-up time of 3.3 years, the recur-rence and progression-free survival rates were similar in the two groups. The incidence of wound complications was higher in the preoperative group versus the postoperative group (35% vs. 17%), and the incidence of wound complications was |
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