Current Problems in Surgery
Volume 46, Issue 6 , Pages 445-503 , June 2009

Retroperitoneal and Intra-Abdominal Sarcoma

  • J. Spencer Liles, MD

      Affiliations

    • Resident in General Surgery, University of Alabama at Birmingham, Birmingham, Alabama
  • ,
  • Ching-Wei D. Tzeng, MD

      Affiliations

    • Resident in General Surgery, University of Alabama at Birmingham, Birmingham, Alabama
  • ,
  • Joshua J. Short, MD, PhD

      Affiliations

    • Resident in General Surgery, University of Alabama at Birmingham, Birmingham, Alabama
  • ,
  • Peter Kulesza, MD, PhD

      Affiliations

    • Assistant Professor of Pathology and Associate Scientist, University of Alabama at Birmingham, Birmingham, Alabama
  • ,
  • Martin J. Heslin, MD

      Affiliations

    • Professor of Surgery, Chief of Surgical Oncology, Director, Multidisciplinary GI Oncology Clinic, Senior Associate Chief of Staff, Associate Director, UAB Comprehensive Cancer Center, University of Alabama at Birmingham, Birmingham, Alabama

References 

  1. Singer S, Eberlein TJ. Surgical management of soft-tissue sarcoma. In:  Cameron JL editors. Advances in Surgery. Vol. 31:St. Louis: Mosby-Year Book, Inc; 1998;p. 395–420
  2. Jemal A, Siegel R, Ward E, Hao Y, Xu J, Murray T, et al. Cancer statistics, 2008. CA Cancer J Clin. 2008;58:71–96
  3. Demetri GD, Benjamin RS, Blanke CD, Blay JY, Casali P, Choi H, et al. NCCN Task Force report: management of patients with gastrointestinal stromal tumor (GIST)—update of the NCCN clinical practice guidelines. J Natl Compr Canc Netw. 2007;5(suppl 2):S1–S29quiz S30
  4. Mann GB, Lewis JJ, Brennan MF. Adult soft tissue sarcoma. Aust N Z J Surg. 1999;69:336–343
  5. Skubitz KM, D'Adamo DR. Sarcoma. Mayo Clin Proc. 2007;82:1409–1432
  6. Gutierrez JC, Perez EA, Franceschi D, Moffat FL, Livingstone AS, Koniaris LG. Outcomes for soft-tissue sarcoma in 8249 cases from a large state cancer registry. J Surg Res. 2007;141:105–114
  7. Stoeckle E, Coindre JM, Bonvalot S, Kantor G, Terrier P, Bonichon F, et al. Prognostic factors in retroperitoneal sarcoma: a multivariate analysis of a series of 165 patients of the French Cancer Center Federation Sarcoma Group. Cancer. 2001;92:359–368
  8. Jaques DP, Coit DG, Hajdu SI, Brennan MF. Management of primary and recurrent soft-tissue sarcoma of the retroperitoneum. Ann Surg. 1990;212:51–59
  9. Porter GA, Baxter NN, Pisters PW. Retroperitoneal sarcoma: a population-based analysis of epidemiology, surgery, and radiotherapy. Cancer. 2006;106:1610–1616
  10. Lewis JJ, Leung D, Woodruff JM, Brennan MF. Retroperitoneal soft-tissue sarcoma: analysis of 500 patients treated and followed at a single institution. Ann Surg. 1998;228:355–365
  11. Gronchi A, Casali PG, Fiore M, Mariani L, Lo Vullo S, Bertulli R, et al. Retroperitoneal soft tissue sarcomas: patterns of recurrence in 167 patients treated at a single institution. Cancer. 2004;100:2448–2455
  12. Kilkenny JW, Bland KI, Copeland EM. Retroperitoneal sarcoma: the University of Florida experience. J Am Coll Surg. 1996;182:329–339
  13. Pirayesh A, Chee Y, Helliwell TR, Hershman MJ, Leinster SJ, Fordham MV, et al. The management of retroperitoneal soft tissue sarcoma: a single institution experience with a review of the literature. Eur J Surg Oncol. 2001;27:491–497
  14. McGrath PC, Neifeld JP, Lawrence W, DeMay RM, Kay S, Horsley JS, et al. Improved survival following complete excision of retroperitoneal sarcomas. Ann Surg. 1984;200:200–204
  15. Mack TM. Sarcomas and other malignancies of soft tissue, retroperitoneum, peritoneum, pleura, heart, mediastinum, and spleen. Cancer. 1995;75(1 suppl):211–244
  16. Storm FK, Mahvi DM. Diagnosis and management of retroperitoneal soft-tissue sarcoma. Ann Surg. 1991;214:2–10
  17. Eilber FC, Eilber KS, Eilber FR. Retroperitoneal sarcomas. Curr Treat Options Oncol. 2000;1:274–278
  18. Felix EL, Wood DK, Das Gupta TK. Tumors of the retroperitoneum. Curr Prob Cancer. 1981;6:1–47
  19. Kutta A, Engelmann U, Schmidt U, Senge T. Primary retroperitoneal tumors. Urol Int. 1992;48:353–357
  20. Storm FK, Eilber FR, Mirra J, Morton DL. Retroperitoneal sarcomas: a reappraisal of treatment. J Surg Oncol. 1981;17:1–7
  21. Van Dam PA, Lowe DG, McKenzie-Gray B, Shepherd JH. Retroperitoneal soft tissue sarcomas: a review of the literature. Obstet Gynecol Surv. 1990;45:670–682
  22. Van Roggen JF, Hogendoorn PC. Soft tissue tumours of the retroperitoneum. Sarcoma. 2000;4:17–26
  23. McCallum OJ, Burke 2nd JJ, Childs AJ, Ferro A, Gallup DG. Retroperitoneal liposarcoma weighing over one hundred pounds with review of the literature. Gynecol Oncol. 2006;103:1152–1154
  24. Lewis JJ, Brennan MF. The management of retroperitoneal soft tissue sarcoma. Adv Surg. 1999;33:329–344
  25. Cheifetz R, Catton CN, Kandel R, O'Sullivan B, Couture J, Swallow CJ. Recent progress in the management of retroperitoneal sarcoma. Sarcoma. 2001;5:17–26
  26. Karakousis CP, Velez AF, Emrich LJ. Management of retroperitoneal sarcomas and patient survival. Am J Surg. 1985;150:376–380
  27. Zornig C, Weh HJ, Krull A, Schwarz R, Dieckmann J, Rehpenning W, et al. Retroperitoneal sarcoma in a series of 51 adults. Eur J Surg Oncol. 1992;18:475–480
  28. van Dalen T, Plooij JM, van Coevorden F, van Geel AN, Hoekstra HJ, Albus-Lutter C, et al. Long-term prognosis of primary retroperitoneal soft tissue sarcoma. Eur J Surg Oncol. 2007;33:234–238
  29. Catton CN, O'Sullivan B, Kotwall C, Cummings B, Hao Y, Fornasier V. Outcome and prognosis in retroperitoneal soft tissue sarcoma. Int J Radiat Oncol Biol Phys. 1994;29:1005–1010
  30. Pierie JP, Betensky RA, Choudry U, Willet CG, Souba WW, Ott MJ. Outcomes in a series of 103 retroperitoneal sarcomas. Eur J Surg Oncol. 2006;32:1235–1241
  31. Dalton RR, Donohue JH, Mucha P, van Heerden JA, Reiman HM, Chen SP. Management of retroperitoneal sarcomas. Surgery. 1989;106:725–732discussion 732-3
  32. Heslin MJ, Lewis JJ, Nadler E, Newman E, Woodruff JM, Casper ES, et al. Prognostic factors associated with long-term survival for retroperitoneal sarcoma: implications for management. J Clin Oncol. 1997;15:2832–2839
  33. Goffman T, Tochner Z, Glatstein E. Primary treatment of large and massive adult sarcomas with iododeoxyuridine and aggressive hyperfractionated irradiation. Cancer. 1991;67:572–576
  34. Fletcher CD. The evolving classification of soft tissue tumours: an update based on the new WHO classification. Histopathology. 2006;48:3–12
  35. Cody HS, Turnbull AD, Fortner JG, Hadju SI. The continuing challenge of retroperitoneal sarcomas. Cancer. 1981;47:2147–2152
  36. Pack GT, Tabah EJ. Primary retroperitoneal tumors: a study of 120 cases. Int Abstr Surg. 1954;99:313–341
  37. O'Brien JE, Stout AP. Malignant fibrous xanthomas. Cancer. 1964;17:1445–1455
  38. Rooser B, Willen H, Gustafson P, Alvegard TA, Rydholm A. Malignant fibrous histiocytoma of soft tissue (A population-based epidemiologic and prognostic study of 137 patients). Cancer. 1991;67:499–505
  39. Enzinger FM. Malignant fibrous histiocytoma 20 years after Stout. Am J Surg Pathol. 1986;10(suppl 1):43–53
  40. Fletcher CD. Pleomorphic malignant fibrous histiocytoma: fact or fiction? (A critical reappraisal based on 159 tumors diagnosed as pleomorphic sarcoma). Am J Surg Pathol. 1992;16:213–228
  41. Coindre JM, Mariani O, Chibon F, Mairal A, de Saint Aubain Somerhausen N, Favre-Guillevin E, et al. Most malignant fibrous histiocytomas developed in the retroperitoneum are dedifferentiated liposarcomas: a review of 25 cases initially diagnosed as malignant fibrous histiocytoma. Mod Pathol. 2003;16:256–262
  42. Fabre-Guillevin E, Coindre JM, de Saint Aubain Somerhausen N, Bonichon F, Stoeckle E, Bui NB, et al. Retroperitoneal liposarcomas: follow-up analysis of dedifferentiation after clinicopathologic reexamination of 86 liposarcomas and malignant fibrous histiocytomas. Cancer. 2006;106:2725–2733
  43. Chibon F, Mariani O, Derre J, Malinge S, Coindre JM, Guillou L, et al. A subgroup of malignant fibrous histiocytomas is associated with genetic changes similar to those of well-differentiated liposarcomas. Cancer Genet Cytogenet. 2002;139:24–29
  44. Daugaard S. Current soft-tissue sarcoma classifications. Eur J Cancer. 2004;40:543–548
  45. Francis IR, Cohan RH, Varma DG, Sondak VK. Retroperitoneal sarcomas. Cancer Imaging. 2005;5:89–94
  46. Dalal KM, Kattan MW, Antonescu CR, Brennan MF, Singer S. Subtype specific prognostic nomogram for patients with primary liposarcoma of the retroperitoneum, extremity, or trunk. Ann Surg. 2006;244:381–391
  47. Mentzel T, Fletcher CD. Lipomatous tumours of soft tissues: an update. Virchows Arch. 1995;427:353–363
  48. Singer S, Antonescu CR, Riedel E, Brennan MF. Histologic subtype and margin of resection predict pattern of recurrence and survival for retroperitoneal liposarcoma. Ann Surg. 2003;238:358–370discussion 370-1
  49. Evans HL. Liposarcoma: a study of 55 cases with a reassessment of its classification. Am J Surg Pathol. 1979;3:507–523
  50. Hostein I, Pelmus M, Aurias A, Pedeutour F, Mathoulin-Pelissier S, Coindre JM. Evaluation of MDM2 and CDK4 amplification by real-time PCR on paraffin wax-embedded material: a potential tool for the diagnosis of atypical lipomatous tumours/well-differentiated liposarcomas. J Pathol. 2004;202:95–102
  51. Weiss SW, Rao VK. Well-differentiated liposarcoma (atypical lipoma) of deep soft tissue of the extremities, retroperitoneum, and miscellaneous sites (A follow-up study of 92 cases with analysis of the incidence of “dedifferentiation”). Am J Surg Pathol. 1992;16:1051–1058
  52. Henricks WH, Chu YC, Goldblum JR, Weiss SW. Dedifferentiated liposarcoma: a clinicopathological analysis of 155 cases with a proposal for an expanded definition of dedifferentiation. Am J Surg Pathol. 1997;21:271–281
  53. Lahat G, Tuvin D, Wei C, Anaya DA, Bekele BN, Lazar AJ. New perspectives for staging and prognosis in soft tissue sarcoma. Ann Surg Oncol. 2008;15:2739–2748
  54. Hashimoto H, Tsuneyoshi M, Enjoji M. Malignant smooth muscle tumors of the retroperitoneum and mesentery: a clinicopathologic analysis of 44 cases. J Surg Oncol. 1985;28:177–186
  55. Weiss SW. Smooth muscle tumors of soft tissue. Adv Anat Pathol. 2002;9:351–359
  56. Shmookler BM, Lauer DH. Retroperitoneal leiomyosarcoma (A clinicopathologic analysis of 36 cases). Am J Surg Pathol. 1983;7:269–280
  57. Hartman DS, Hayes WS, Choyke PL, Tibbetts GP. From the archives of the AFIP (Leiomyosarcoma of the retroperitoneum and inferior vena cava: radiologic-pathologic correlation). Radiographics. 1992;12:1203–1220
  58. Rajani B, Smith TA, Reith JD, Goldblum JR. Retroperitoneal leiomyosarcomas unassociated with the gastrointestinal tract: a clinicopathologic analysis of 17 cases. Mod Pathol. 1999;12:21–28
  59. Shindo S, Matsumoto H, Ogata K, Katahira S, Kojima A, Iyori K, et al. Surgical treatment of retroperitoneal leiomyosarcoma invading the inferior vena cava: report of three cases. Surg Today. 2002;32:929–933
  60. Hassan I, Park SZ, Donohue JH, Nagorney DM, Kay PA, Nasciemento AG, et al. Operative management of primary retroperitoneal sarcomas: a reappraisal of an institutional experience. Ann Surg. 2004;239:244–250
  61. Ranchod M, Kempson RL. Smooth muscle tumors of the gastrointestinal tract and retroperitoneum: a pathologic analysis of 100 cases. Cancer. 1977;39:255–262
  62. das Gupta TK, Brasfield RD. Solitary malignant schwannoma. Ann Surg. 1970;171:419–428
  63. Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve sheath tumors (A clinicopathologic study of 120 cases). Cancer. 1986;57:2006–2021
  64. Fletcher CDM, Unni KK, Mertens F. Pathology and Genetics of Tumours of Soft Tissue and Bone (World Health Organization classification of tumours). Lyon: IARC Press; 2002;
  65. Arnoletti P, Jhala N. Retroperitoneal hemangiopericytoma. J Am Coll Surg. 2003;197:687–688
  66. Espat NJ, Lewis JJ, Leung D, Woodruff JM, Antonescu CR, Shia J. Conventional hemangiopericytoma: modern analysis of outcome. Cancer. 2002;95:1746–1751
  67. Doglietto GB, Tortorelli AP, Papa V, Rosa F, Bossola M, Prete FP, et al. Giant retroperitoneal sarcomas: a single institution experience. World J Surg. 2007;31:1047–1054
  68. Spillane AJ. Retroperitoneal sarcoma: time for a change in attitude?. ANZ J Surg. 2001;71:303–308
  69. Windham TC, Pisters PW. Retroperitoneal sarcomas. Cancer Control. 2005;12:36–43
  70. Feig BW. Retroperitoneal sarcomas. Surg Oncol Clin N Am. 2003;12:369–377
  71. Stower MJ, Hardcastle JD. Malignant retroperitoneal sarcoma: a review of 32 cases. Clin Oncol. 1982;8:257–263
  72. Woodall CE, Scoggins CR. Retroperitoneal and visceral sarcomas: issues for the general surgeon. Am Surg. 2007;73:631–635
  73. Wist E, Solheim OP, Jacobsen AB, Blom P. Primary retroperitoneal sarcomas (A review of 36 cases). Acta Radiol Oncol. 1985;24:305–310
  74. Sarma DP, Weilbaecher TG. Retroperitoneal malignant fibrous histiocytoma presenting with inferior vena caval obstruction. J Surg Oncol. 1986;32:153–155
  75. Bolin TE, Bolin SG, Wetterfors J. Retroperitoneal sarcomas (An analysis of 32 cases). Acta Chir Scand. 1988;154:627–629
  76. Salvadori B, Cusumano F, Delledonne V, De Lellis R, Conti R. Surgical treatment of 43 retroperitoneal sarcomas. Eur J Surg Oncol. 1986;12:29–33
  77. Eddleston JM. Pyrexia associated with a retroperitoneal tumour. Anaesthesia. 1987;42:213–214
  78. Makela J, Kiviniemi H, Laitinen S. Prognostic factors predicting survival in the treatment of retroperitoneal sarcoma. Eur J Surg Oncol. 2000;26:552–555
  79. Lucey C, Graham WJ. Hypoglycaemia due to retroperitoneal sarcoma with insulin-like activity. Ir J Med Sci. 1968;7:413–422
  80. Newlon JL, Couch M, Brennan J. Castleman's disease: three case reports and a review of the literature. Ear Nose Throat J. 2007;86:414–418
  81. Meador TL, McLarney JK. CT features of Castleman disease of the abdomen and pelvis. AJR Am J Roentgenol. 2000;175:115–118
  82. Cronin CG, Lohan DG, Blake MA, Roche C, McCarthy P, Murphy JM. Retroperitoneal fibrosis: a review of clinical features and imaging findings. AJR Am J Roentgenol. 2008;191:423–431
  83. Lichon FS, Sequeira W, Pilloff A, Skosey JL. Retroperitoneal fibrosis associated with systemic lupus erythematosus: a case report and brief review. J Rheumatol. 1984;11:373–374
  84. Geoghegan T, Byrne AT, Benfayed W, McAuley G, Torreggiani WC. Imaging and intervention of retroperitoneal fibrosis. Australas Radiol. 2007;51:26–34
  85. van Dalen T, Hoekstra HJ, van Geel AN, van Coevorden F, Albus-Lutter C, Slootweg PJ, et al. Locoregional recurrence of retroperitoneal soft tissue sarcoma: second chance of cure for selected patients. Eur J Surg Oncol. 2001;27:564–568
  86. Hueman MT, Herman JM, Ahuja N. Management of retroperitoneal sarcomas. Surg Clin North Am. 2008;88:583–597vii
  87. Heslin MJ, Smith JK. Imaging of soft tissue sarcomas. Surg Oncol Clin N Am. 1999;8:91–107
  88. Tzeng CW, Smith JK, Heslin MJ. Soft tissue sarcoma: preoperative and postoperative imaging for staging. Surg Oncol Clin N Am. 2007;16:389–402
  89. Flechon A, Rivoire M, Droz JP. Management of advanced germ-cell tumors of the testis. Nat Clin Pract Urol. 2008;5:262–276
  90. Sohaib SA, Koh DM, Husband JE. The role of imaging in the diagnosis, staging, and management of testicular cancer. AJR Am J Roentgenol. 2008;191:387–395
  91. Nishino M, Hayakawa K, Minami M, Yamamoto A, Ueda H, Takasu K. Primary retroperitoneal neoplasms: CT and MR imaging findings with anatomic and pathologic diagnostic clues. Radiographics. 2003;23:45–57
  92. Fultz PJ, Hampton WR, Skucas J, Sickel JZ. Differential diagnosis of fat-containing lesions with abdominal and pelvic CT. Radiographics. 1993;13:1265–1280
  93. Chew C, Reid R, O'Dwyer PJ. Value of biopsy in the assessment of a retroperitoneal mass. Surgeon. 2006;4:79–81
  94. Swallow CJ, Catton CN. Local management of adult soft tissue sarcomas. Semin Oncol. 2007;34:256–269
  95. van Dalen T, Hennipman A, van Coevorden F, Hoekstra HJ, van Geel BN, Slootweg P, et al. Evaluation of a clinically applicable post-surgical classification system for primary retroperitoneal soft-tissue sarcoma. Ann Surg Oncol. 2004;11:483–490
  96. Mendenhall WM, Zlotecki RA, Hochwald SN, Hemming AW, Grobmyer SR, Cance WG. Retroperitoneal soft tissue sarcoma. Cancer. 2005;104:669–675
  97. Chen CQ, Yin L, Peng CH, Cai Y, Li YF, Zhao R, et al. Prognostic factors of retroperitoneal soft tissue sarcomas: analysis of 132 cases. Chin Med J (Engl). 2007;120:1047–1050
  98. Raut CP, Pisters PW. Retroperitoneal sarcomas: combined-modality treatment approaches. J Surg Oncol. 2006;94:81–87
  99. Fein DA, Corn BW, Lanciano RM, Herbert SH, Hoffman JP, Coia LR. Management of retroperitoneal sarcomas: does dose escalation impact on locoregional control?. Int J Radiat Oncol Biol Phys. 1995;31:129–134
  100. Sindelar WF, Kinsella TJ, Chen PW, DeLaney TF, Tepper JE, Rosenberg SA, et al. Intraoperative radiotherapy in retroperitoneal sarcomas (Final results of a prospective, randomized, clinical trial). Arch Surg. 1993;128:402–410
  101. Tepper JE, Suit HD, Wood WC, Proppe KH, Harmon D, McNulty P. Radiation therapy of retroperitoneal soft tissue sarcomas. Int J Radiat Oncol Biol Phys. 1984;10:825–830
  102. Kepka L, DeLaney TF, Suit HD, Goldberg SI. Results of radiation therapy for unresected soft-tissue sarcomas. Int J Radiat Oncol Biol Phys. 2005;63:852–859
  103. Tzeng CW, Fiveash JB, Popple RA, Arnoletti JP, Russo SM, Urist MM, et al. Preoperative radiation therapy with selective dose escalation to the margin at risk for retroperitoneal sarcoma. Cancer. 2006;107:371–379
  104. Bobin JY, Al-Lawati T, Granero LE, Adham M, Romestaing P, Chapet O, et al. Surgical management of retroperitoneal sarcomas associated with external and intraoperative electron beam radiotherapy. Eur J Surg Oncol. 2003;29:676–681
  105. Willett CG, Suit HD, Tepper JE, Mankin HJ, Convery K, Rosenberg AL, et al. Intraoperative electron beam radiation therapy for retroperitoneal soft tissue sarcoma. Cancer. 1991;68:278–283
  106. Alektiar KM, Hu K, Anderson L, Brennan MF, Harrison LB, et al. High-dose-rate intraoperative radiation therapy (HDR-IORT) for retroperitoneal sarcomas. Int J Radiat Oncol Biol Phys. 2000;47:157–163
  107. Petersen IA, Haddock MG, Donohue JH, Nagorney DM, Grill JP, Sargent DJ, et al. Use of intraoperative electron beam radiotherapy in the management of retroperitoneal soft tissue sarcomas. Int J Radiat Oncol Biol Phys. 2002;52:469–475
  108. Pisters PW, Ballo MT, Fenstermacher MJ, Feig BW, Hunt KK, Raymond KA, et al. Phase I trial of preoperative concurrent doxorubicin and radiation therapy, surgical resection, and intraoperative electron-beam radiation therapy for patients with localized retroperitoneal sarcoma. J Clin Oncol. 2003;21:3092–3097
  109. Jones JJ, Catton CN, O'Sullivan B, Couture J, Heisler RL, Kandel RA, et al. Initial results of a trial of preoperative external-beam radiation therapy and postoperative brachytherapy for retroperitoneal sarcoma. Ann Surg Oncol. 2002;9:346–354
  110. Zlotecki RA, Katz TS, Morris CG, Lind DS, Hochwald SN. Adjuvant radiation therapy for resectable retroperitoneal soft tissue sarcoma: the University of Florida experience. Am J Clin Oncol. 2005;28:310–316
  111. Gieschen HL, Spiro IJ, Suit HD, Ott MJ, Rattner DW, Ancukiewicz M, et al. Long-term results of intraoperative electron beam radiotherapy for primary and recurrent retroperitoneal soft tissue sarcoma. Int J Radiat Oncol Biol Phys. 2001;50:127–131
  112. Pollack A, Zagars GK, Goswitz MS, Pollock RA, Feig BW, Pisters PW. Preoperative vs. postoperative radiotherapy in the treatment of soft tissue sarcomas: a matter of presentation. Int J Radiat Oncol Biol Phys. 1998;42:563–572
  113. Pawlik TM, Pisters PW, Mikula L, Feig BW, Hunt KK, Cormier JN, et al. Long-term results of two prospective trials of preoperative external beam radiotherapy for localized intermediate- or high-grade retroperitoneal soft tissue sarcoma. Ann Surg Oncol. 2006;13:508–517
  114. Fiveash JB, Hyatt MD, Caranto J, Popple RA, Bland KI, Urist MM, et al. Preoperative IMRT with dose escalation to tumor subvolumes for retroperitoneal sarcomas: initial clinical results and potential for future dose escalation. J Rad Oncol Biol Phys. 2002;54s:140
  115. Musat E, Kantor G, Caron J, Lagarde P, Laharie H, Stoeckle E, et al. Comparison of intensity-modulated postoperative radiotherapy with conventional postoperative conformal radiotherapy for retroperitoneal sarcoma. Cancer Radiother. 2004;8:255–261
  116. Tzeng CW, Fiveash JB, Heslin MJ. Radiation therapy for retroperitoneal sarcoma. Expert Rev Anticancer Ther. 2006;6:1251–1260
  117. Cormier JN, Pollock RE. Soft tissue sarcomas. CA Cancer J Clin. 2004;54:94–109
  118. Gold JS, Dematteo RP. Combined surgical and molecular therapy: the gastrointestinal stromal tumor model. Ann Surg. 2006;244:176–184
  119. Katz SC, DeMatteo RP. Gastrointestinal stromal tumors and leiomyosarcomas. J Surg Oncol. 2008;97:350–359
  120. Hirota S, Isozaki K, Moriyama Y, Hashimoto K, Nishida T, Ishiguro S, et al. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science. 1998;279:577–580
  121. Kindblom LG, Remotti HE, Aldenborg F, Meis-Kindblom JM, et al. Gastrointestinal pacemaker cell tumor (GIPACT): gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal. Am J Pathol. 1998;152:1259–1269
  122. Maeda H, Yamagata A, Nishikawa S, Yoshinaga K, Kobayashi S, Nishi K, et al. Requirement of c-kit for development of intestinal pacemaker system. Development. 1992;116:369–375
  123. Huizinga JD, Huizinga JD, Thuneberg L, Kluppel M, Malysz J, Mikkelsen HB, et al. W/kit gene required for interstitial cells of Cajal and for intestinal pacemaker activity. Nature. 1995;373:347–349
  124. Corless CL, Fletcher JA, Heinrich MC. Biology of gastrointestinal stromal tumors. J Clin Oncol. 2004;22:3813–3825
  125. Fletcher CD, Berman JJ, Corless C, Gorstein F, Lasota J, Longley BJ, et al. Diagnosis of gastrointestinal stromal tumors: A consensus approach. Hum Pathol. 2002;33:459–465
  126. DeMatteo RP, Lewis JJ, Leung D, Mudan SS, Woodruff JM, Brennan MF. Two hundred gastrointestinal stromal tumors: recurrence patterns and prognostic factors for survival. Ann Surg. 2000;231:51–58
  127. Mazur MT, Clark HB. Gastric stromal tumors (Reappraisal of histogenesis). Am J Surg Pathol. 1983;7:507–519
  128. Pidhorecky I, Cheney RT, Kraybill WG, Gibbs JF. Gastrointestinal stromal tumors: current diagnosis, biologic behavior, and management. Ann Surg Oncol. 2000;7:705–712
  129. Herrera GA, Pinto de Moraes H, Grizzle WE, Han SG, et al. Malignant small bowel neoplasm of enteric plexus derivation (plexosarcoma) (Light and electron microscopic study confirming the origin of the neoplasm). Dig Dis Sci. 1984;29:275–284
  130. van de Rijn M, Hendrickson MR, Rouse RV. CD34 expression by gastrointestinal tract stromal tumors. Hum Pathol. 1994;25:766–771
  131. Miettinen M, Virolainen M, Maarit Sarlomo R. Gastrointestinal stromal tumors–value of CD34 antigen in their identification and separation from true leiomyomas and schwannomas. Am J Surg Pathol. 1995;19:207–216
  132. Monihan JM, Carr NJ, Sobin LH. CD34 immunoexpression in stromal tumours of the gastrointestinal tract and in mesenteric fibromatoses. Histopathology. 1994;25:469–473
  133. Scarpa M, Bertin M, Ruffolo C, Polese L, D'Amico DF, Angriman I. A systematic review on the clinical diagnosis of gastrointestinal stromal tumors. J Surg Oncol. 2008;98:384–392
  134. Badalamenti G, Rodolico V, Fulfaro F, Cascio S, Cipolla C, Cicero G, et al. Gastrointestinal stromal tumors (GISTs): focus on histopathological diagnosis and biomolecular features. Ann Oncol. 2007;18(suppl 6):136–140
  135. Miettinen M, Lasota J. Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis. Arch Pathol Lab Med. 2006;130:1466–1478
  136. Miettinen M, Lasota J, Sobin LH. Gastrointestinal stromal tumors of the stomach in children and young adults: a clinicopathologic, immunohistochemical, and molecular genetic study of 44 cases with long-term follow-up and review of the literature. Am J Surg Pathol. 2005;29:1373–1381
  137. Prakash S, Sarran L, Socci N, DeMatteo RP, Eisenstat J, Greco AM, et al. Gastrointestinal stromal tumors in children and young adults: a clinicopathologic, molecular, and genomic study of 15 cases and review of the literature. J Pediatr Hematol Oncol. 2005;27:179–187
  138. Reddy P, Boci K, Charbonneau C. The epidemiologic, health-related quality of life, and economic burden of gastrointestinal stromal tumours. J Clin Pharm Ther. 2007;32:557–565
  139. Nilsson B, Bumming P, Meis-Kindblom JM, Oden A, Dortok A, Gustavsson B, et al. Gastrointestinal stromal tumors: the incidence, prevalence, clinical course, and prognostication in the preimatinib mesylate era–a population-based study in western Sweden. Cancer. 2005;103:821–829
  140. Tryggvason G, Gislason HG, Magnusson MK, Jonasson JG. Gastrointestinal stromal tumors in Iceland, 1990-2003: the icelandic GIST study, a population-based incidence and pathologic risk stratification study. Int J Cancer. 2005;117:289–293
  141. Tzen CY, Wang JH, Huang YJ, Wang MN, Lin PC, Lai GL, et al. Incidence of gastrointestinal stromal tumor: a retrospective study based on immunohistochemical and mutational analyses. Dig Dis Sci. 2007;52:792–797
  142. Tran T, Davila JA, El-Serag HB. The epidemiology of malignant gastrointestinal stromal tumors: an analysis of 1,458 cases from 1992 to 2000. Am J Gastroenterol. 2005;100:162–168
  143. Emory TS, Sobin LH, Lukes L, Lee DH, O'Leary TJ. Prognosis of gastrointestinal smooth-muscle (stromal) tumors: dependence on anatomic site. Am J Surg Pathol. 1999;23:82–87
  144. de Silva CM, Reid R. Gastrointestinal stromal tumors (GIST): C-kit mutations, CD117 expression, differential diagnosis and targeted cancer therapy with imatinib. Pathol Oncol Res. 2003;9:13–19
  145. Miettinen M, Lasota J. Gastrointestinal stromal tumors: pathology and prognosis at different sites. Semin Diagn Pathol. 2006;23:70–83
  146. Reith JD, Goldblum JR, Lyles RH, Weiss SW. Extragastrointestinal (soft tissue) stromal tumors: an analysis of 48 cases with emphasis on histologic predictors of outcome. Mod Pathol. 2000;13:577–585
  147. van der Zwan SM, DeMatteo RP. Gastrointestinal stromal tumor: 5 years later. Cancer. 2005;104:1781–1788
  148. Yamamoto H, Oda Y, Kawaguchi K, Nakamura N, Takahira T, Tamiya S, et al. c-kit and PDGFRA mutations in extragastrointestinal stromal tumor (gastrointestinal stromal tumor of the soft tissue). Am J Surg Pathol. 2004;28:479–488
  149. Parfitt JR, Streutker CJ, Riddell RH, Driman DK. Gastrointestinal stromal tumors: a contemporary review. Pathol Res Pract. 2006;202:837–847
  150. Mucciarini C, Rossi G, Bertolini F, Valli R, Cirilli C, Rashid I, et al. Incidence and clinicopathologic features of gastrointestinal stromal tumors (A population-based study). BMC Cancer. 2007;7:230
  151. Hassan I, You YN, Shyyan R, Dozois EJ, Smyrk TC, Okuno SH, et al. Surgically managed gastrointestinal stromal tumors: a comparative and prognostic analysis. Ann Surg Oncol. 2008;15:52–59
  152. Burkill GJ, Badran M, Al-Muderis O, Meirion Thomas J, Judson IR, Fisher C, et al. Malignant gastrointestinal stromal tumor: distribution, imaging features, and pattern of metastatic spread. Radiology. 2003;226:527–532
  153. Levy AD, Remotti HE, Thompson WM, Sobin LH, Miettinen M. Gastrointestinal stromal tumors: radiologic features with pathologic correlation. Radiographics. 2003;23:283–304456; quiz 532
  154. Hueman MT, Schulick RD. Management of gastrointestinal stromal tumors. Surg Clin North Am. 2008;88:599–614vii
  155. King DM. The radiology of gastrointestinal stromal tumours (GIST). Cancer Imaging. 2005;5:150–156
  156. Ghanem N, Altehoefer C, Furtwangler A, Winterer J, Schafer O, Springer O, et al. Computed tomography in gastrointestinal stromal tumors. Eur Radiol. 2003;13:1669–1678
  157. Gayed I, Vu T, Iyer R, Johnson M, Macapinlac H, Swanston N, et al. The role of 18F-FDG PET in staging and early prediction of response to therapy of recurrent gastrointestinal stromal tumors. J Nucl Med. 2004;45:17–21
  158. Cichoz-Lach H, Kasztelan-Szczerbinska B, Slomka M. Gastrointestinal stromal tumors: epidemiology, clinical picture, diagnosis, prognosis and treatment. Pol Arch Med Wewn. 2008;118:216–221
  159. Nickl N. Endoscopic approach to gastrointestinal stromal tumors. Gastrointest Endosc Clin N Am. 2005;15:455–466viii
  160. Fu K, Eloubeidi MA, Jhala NC, Jhala D, Chhieng DC, Eltoum IE. Diagnosis of gastrointestinal stromal tumor by endoscopic ultrasound-guided fine needle aspiration biopsy–a potential pitfall. Ann Diagn Pathol. 2002;6:294–301
  161. Kitamura Y, Hirotab S. Kit as a human oncogenic tyrosine kinase. Cell Mol Life Sci. 2004;61:2924–2931
  162. Duensing A, Medeiros F, McConarty B, Joseph NE, Panigrahy D, Singer S, et al. Mechanisms of oncogenic KIT signal transduction in primary gastrointestinal stromal tumors (GISTs). Oncogene. 2004;23:3999–4006
  163. Hornick JL, Fletcher CD. The role of KIT in the management of patients with gastrointestinal stromal tumors. Hum Pathol. 2007;38:679–687
  164. Sommer G, Agosti V, Ehlers I, Rossi F, Corbacioglu S, Farkas J, et al. Gastrointestinal stromal tumors in a mouse model by targeted mutation of the Kit receptor tyrosine kinase. Proc Natl Acad Sci U S A. 2003;100:6706–6711
  165. Druker BJ, Tamura S, Buchdunger E, Ohno S, Segal GM, Fanning S, et al. Effects of a selective inhibitor of the Abl tyrosine kinase on the growth of Bcr-Abl positive cells. Nat Med. 1996;2:561–566
  166. Buchdunger E, Cioffi CL, Law N, Stover D, Ohno-Jones S, Druker BJ, et al. Abl protein-tyrosine kinase inhibitor STI571 inhibits in vitro signal transduction mediated by c-kit and platelet-derived growth factor receptors. J Pharmacol Exp Ther. 2000;295:139–145
  167. Heinrich MC, Griffith DJ, Druker BJ, Wait CL, Ott KA, Zigler AJ. Inhibition of c-kit receptor tyrosine kinase activity by STI 571, a selective tyrosine kinase inhibitor. Blood. 2000;96:925–932
  168. Joensuu H, Roberts PJ, Sarlomo-Rikala M, Andersson LC, Tervahartiala P, Tuveson D, et al. Effect of the tyrosine kinase inhibitor STI571 in a patient with a metastatic gastrointestinal stromal tumor. N Engl J Med. 2001;344:1052–1056
  169. Debiec-Rychter M, Sciot R, Le Cesne A, Schlemmer M, Hohenberger P, van Oosterom AT, et al. KIT mutations and dose selection for imatinib in patients with advanced gastrointestinal stromal tumours. Eur J Cancer. 2006;42:1093–1103
  170. Corless CL, McGreevey L, Haley A, Town A, Heinrich MC. KIT mutations are common in incidental gastrointestinal stromal tumors one centimeter or less in size. Am J Pathol. 2002;160:1567–1572
  171. Heinrich MC, Corless CL, Demetri GD, Blanke CD, von Mehren M, Joensuu H, et al. Kinase mutations and imatinib response in patients with metastatic gastrointestinal stromal tumor. J Clin Oncol. 2003;21:4342–4349
  172. Rubin BP, Singer S, Tsao C, Duensing A, Lux ML, Ruiz R, et al. KIT activation is a ubiquitous feature of gastrointestinal stromal tumors. Cancer Res. 2001;61:8118–8121
  173. Antonescu CR, Sommer G, Sarran L, Tschernyavsky SJ, Riedel E, Woodruff JM, et al. Association of KIT exon 9 mutations with nongastric primary site and aggressive behavior: KIT mutation analysis and clinical correlates of 120 gastrointestinal stromal tumors. Clin Cancer Res. 2003;9:3329–3337
  174. Lasota J, Wozniak A, Sarlomo-Rikala M, Rys J, Kordek R, Nassar A, et al. Mutations in exons 9 and 13 of KIT gene are rare events in gastrointestinal stromal tumors (A study of 200 cases). Am J Pathol. 2000;157:1091–1095
  175. Lasota J, Corless CL, Heinrich MC, Debiec-Rychter , Sciot R, Wardelmann E, et al. Clinicopathologic profile of gastrointestinal stromal tumors (GISTs) with primary KIT exon 13 or exon 17 mutations: a multicenter study on 54 cases. Mod Pathol. 2008;21:476–484
  176. Wardelmann E, Losen I, Hans V, Neidt I, Speidel N, Bierhoff E, et al. Deletion of Trp-557 and Lys-558 in the juxtamembrane domain of the c-kit protooncogene is associated with metastatic behavior of gastrointestinal stromal tumors. Int J Cancer. 2003;106:887–895
  177. Andersson J, Bumming P, Meis-Kindblom JM, Sihto H, Nupponen N, Joensuu H, et al. Gastrointestinal stromal tumors with KIT exon 11 deletions are associated with poor prognosis. Gastroenterology. 2006;130:1573–1581
  178. Martin J, Poveda A, Llombart-Bosch A, Ramos R, Lopez-Guerrero JA, Garcia del Muro J, et al. Deletions affecting codons 557-558 of the c-KIT gene indicate a poor prognosis in patients with completely resected gastrointestinal stromal tumors: a study by the Spanish Group for Sarcoma Research (GEIS). J Clin Oncol. 2005;23:6190–6198
  179. Heinrich MC, Corless CL, Duensing A, McGreevey L, Chen CJ, Joseph N, et al. PDGFRA activating mutations in gastrointestinal stromal tumors. Science. 2003;299:708–710
  180. Lasota J, Dansonka-Mieszkowska A, Sobin LH, Miettinen M. A great majority of GISTs with PDGFRA mutations represent gastric tumors of low or no malignant potential. Lab Invest. 2004;84:874–883
  181. Wardelmann E, Hrychyk A, Merkelbach-Bruse S, Pauls K, Goldstein J, Hohenberger P, et al. Association of platelet-derived growth factor receptor alpha mutations with gastric primary site and epithelioid or mixed cell morphology in gastrointestinal stromal tumors. J Mol Diagn. 2004;6:197–204
  182. Gold JS, van der Zwan SM, Gonen M, Maki RG, Singer S, Brennan MF, et al. Outcome of metastatic GIST in the era before tyrosine kinase inhibitors. Ann Surg Oncol. 2007;14:134–142
  183. Verweij J, Casali PG, Zalcberg J, LeCesne A, Reichardt P, Blay JY, et al. Progression-free survival in gastrointestinal stromal tumours with high-dose imatinib: randomised trial. Lancet. 2004;364:1127–1134
  184. Blanke CD, Demetri GD, von Mehren M, Heinrich MC, Eisenberg B, Fletcher JA, et al. Long-term results from a randomized phase II trial of standard- versus higher-dose imatinib mesylate for patients with unresectable or metastatic gastrointestinal stromal tumors expressing KIT. J Clin Oncol. 2008;26:620–625
  185. Rutkowski P, Nowecki ZI, Michej W, Debiec-Rychter M, Wozniak A, Limon J, et al. Risk criteria and prognostic factors for predicting recurrences after resection of primary gastrointestinal stromal tumor. Ann Surg Oncol. 2007;14:2018–2027
  186. Takahashi T, Nakajima K, Nishitani A, Souma Y, Hirota S, Sawa Y, et al. An enhanced risk-group stratification system for more practical prognostication of clinically malignant gastrointestinal stromal tumors. Int J Clin Oncol. 2007;12:369–374
  187. Raut CP, DeMatteo RP. Prognostic factors for primary GIST: prime time for personalized therapy?. Ann Surg Oncol. 2008;15:4–6
  188. Singer S, Rubin BP, Lux ML, Chen CJ, Demetri GD, Fletcher CD, et al. Prognostic value of KIT mutation type, mitotic activity, and histologic subtype in gastrointestinal stromal tumors. J Clin Oncol. 2002;20:3898–3905
  189. Langer C, Gunawan B, Schuler P, Huber W, Fuzesi L, Becker H, et al. Prognostic factors influencing surgical management and outcome of gastrointestinal stromal tumours. Br J Surg. 2003;90:332–339
  190. Dematteo RP, Gold JS, Saran L, Gonen M, Liau KH, Maki RG, et al. Tumor mitotic rate, size, and location independently predict recurrence after resection of primary gastrointestinal stromal tumor (GIST). Cancer. 2008;112:608–615
  191. Kim TW, Lee H, Kang YK, Choe MS, Ryu MH, Chang HM, et al. Prognostic significance of c-kit mutation in localized gastrointestinal stromal tumors. Clin Cancer Res. 2004;10:3076–3081
  192. Ballarini C, Intra M, Ceretti AP, Prestipino F, Bianchi FM, Sparacio F, et al. Gastrointestinal stromal tumors: a “benign” tumor with hepatic metastasis after 11 years. Tumori. 1998;84:78–81
  193. Joensuu H. Risk stratification of patients diagnosed with gastrointestinal stromal tumor. Hum Pathol. 2008;39:1411–1419
  194. Miettinen M, Makhlouf HR, Sobin LH, Lasota J. Gastrointestinal stromal tumors (GISTs) of the jejunum and ileum: a clinicopathologic, immunohistochemical and molecular genetic study of 906 cases prior to imatinib with long-term follow-up. Am J Surg Pathol. 2006;30:477–489
  195. Joensuu H. Sunitinib for imatinib-resistant GIST. Lancet. 2006;368:1303–1304
  196. Antonescu CR, Besmer P, Guo T, Arkun K, Hom G, Koryotowski B, et al. Acquired resistance to imatinib in gastrointestinal stromal tumor occurs through secondary gene mutation. Clin Cancer Res. 2005;11:4182–4190
  197. Demetri GD, van Oosterom AT, Garrett CR, Blackstein ME, Shah MH, Verweij J, et al. Efficacy and safety of sunitinib in patients with advanced gastrointestinal stromal tumour after failure of imatinib: a randomised controlled trial. Lancet. 2006;368:1329–1338
  198. Goodman VL, Rock EP, Dagher R, Ramchandani RP, Abraham S, Gobburu JV, et al. Approval summary: sunitinib for the treatment of imatinib refractory or intolerant gastrointestinal stromal tumors and advanced renal cell carcinoma. Clin Cancer Res. 2007;13:1367–1373
  199. DeMatteo RP, Owzar K, Antonescu C, Maki R, Demetri GD, McCarter M, et al. Efficacy of adjuvant imatinib mesylate following complete resection of localized, primary gastrointestinal stromal tumor (GIST) at high risk of recurrence: the U.S. Intergroup Phase II Trial ACOSOG Z9000. 2008;Oral Presentation at the 2008 Gastrointestinal Cancers Symposium, Orlando, Florida, January 25-27
  200. An JY, Heo JS, Noh JH, Sohn TS, Nam SJ, Choi SH, et al. Primary malignant retroperitoneal tumors: analysis of a single institutional experience. Eur J Surg Oncol. 2007;33:376–382
  201. Kairaluoma MI, Krause-Makitalo B, Pokela R, Stahlberg M, Laitinen S, Mokka RE. Primary retroperitoneal tumours in adults. Ann Chir Gynaecol. 1984;73:313–318
  202. Xu YH, Guo KJ, Guo RX, Ge CL, Tian YL, He SG. Surgical management of 143 patients with adult primary retroperitoneal tumor. World J Gastroenterol. 2007;13:2619–2621
  203. In:  Greene FL,  Page DL,  Fleming ID,  Fritz A,  Balch CM,  Haller DG,  Morrow M editor. AJCC Cancer Staging Manual. (ed 6). New York: Springer; 2002;

PII: S0011-3840(09)00005-7

doi: 10.1067/j.cpsurg.2009.01.004

Current Problems in Surgery
Volume 46, Issue 6 , Pages 445-503 , June 2009