Annals of Surgical Oncology

, Volume 8, Issue 1, pp 50–59 | Cite as

Malignant Gastrointestinal Stromal Tumors of the Small Intestine: A Review of 50 Cases From a Prospective Database

  • Jacqueline A. Crosby
  • Charles N. Catton
  • Aileen Davis
  • Jean Couture
  • Brian O’Sullivan
  • Rita Kandel
  • Carol J. Swallow
Original Article


Background: Malignant gastrointestinal stromal tumors (M-GIST) are rare mesenchymal tumors originating in the wall of the gastrointestinal (GI) tract. Previous studies have included limited numbers of patients, and most included malignant and benign cases from throughout the GI tract. We reviewed the experience of a single tertiary cancer care center with M-GIST of the small intestine only.

Methods: A prospective database identified all patients seen from 1989 to 1998. Clinical and pathological data, treatment, and outcome were analyzed. Overall median follow-up time was 24 months (range, 1-176 months).

Results: Fifty patients (31 male, 19 female) were identified. Mean age at diagnosis was 55 years. Disease was localized in 11 patients, locally advanced (invasion into adjacent organs/peritoneum) in 24 patients, perforated in 4 patients, multiple primary lesions in 2 patients, and distant metastases in 9 patients. All patients underwent resection, which was complete in 70%. Locoregional recurrence (LR) developed in 43% (median, 25 months), and distant metastases in 59% (median, 21 months) of patients at risk. At last follow-up, 14 patients were alive (6 disease-free), 2 had died disease-free, and 34 died with recurrent disease. Overall survival (OS) was similar for localized and locally advanced disease; OS also was similar for patients with multiple primaries and distant metastases at diagnosis. Patients were grouped into three stages: (I) patients with localized and locally advanced disease; (II) patients with perforated; and (III) patients with multiple primaries and distant metastases. Actuarial OS at 5 years was 41% (n = 50)—42% for those with complete resection and 8% for incomplete resection. Univariable analysis showed that earlier stage at diagnosis (P = .001) and completeness of resection (P = .004) predicted for longer OS.

Conclusions: Most patients with M-GIST of the small intestine relapse following resection, but survival may be prolonged. In univariable analysis, stage at presentation and complete resection were significant prognostic variables for OS; grade was not significant. Localized and locally advanced M-GIST of the small intestine have a mean OS > 5 years. Complete resection should be the goal of initial surgical treatment.

Key Words

GIST Gastrointestinal stromal tumors Intestinal sarcoma Small intestinal neoplasms 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Lewis JJ, Brennan MF. Soft tissue sarcomas. Curr Probl Surg 1996; 33: 817–72.PubMedGoogle Scholar
  2. 2.
    Mazur MT, Clark HB. Gastric stromal tumors: Reappraisal of histogenesis. Am J Surg Pathol 1983; 7: 507–19.PubMedGoogle Scholar
  3. 3.
    Franquemont DW. Differentiation and risk assessment of gastrointestinal stromal tumors. Am J Clin Pathol 1995; 103: 41–7.PubMedGoogle Scholar
  4. 4.
    Suster S. Gastrointestinal stromal tumors. Semin Diagn Pathol 1996; 13: 297–313.PubMedGoogle Scholar
  5. 5.
    Kindblom LG, Remotti HE, Aldenborg F, Meis-Kindblom JM. Gastrointestinal pacemaker cell tumor (GIPACT). Gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal. Am J Pathol 1998; 152: 1259–69.PubMedGoogle Scholar
  6. 6.
    Chan JKC. Mesenchymal tumors of the gastrointestinal tract: a paradise for acronyms (STUMP, GIST, GANT, and now GIPACT), implication of c-kit in genesis, and yet another of the many emerging roles of the interstitial cell of Cajal in the pathogenesis of gastrointestinal diseases? Adv Anat Pathol 1999; 6: 19–40.PubMedCrossRefGoogle Scholar
  7. 7.
    Sakurai S, Fukasawa T, Chong JM, Tanaka A, Fukayama M. Embryonic form of smooth muscle myosin heavy chain (Smemb/MHC-B) in gastrointestinal stromal tumor and interstitial cells of Cajal. Am J Pathol 1999; 154: 23–8.PubMedGoogle Scholar
  8. 8.
    Sircar K, Hewlett BR, Huizinga JD, Chorneyko K, Berezin I, Riddell RH. Interstitial cells of Cajal as precursors of gastrointestinal stromal tumors. Am J Surg Pathol 1999; 23: 377–89.CrossRefPubMedGoogle Scholar
  9. 9.
    Wu SS, Buchmiller TL, Close P, Gershman GB, Peng SK, French SW. Congenital gastrointestinal pacemaker cell tumor. Arch Pathol Lab Med 1999; 123: 842–5.PubMedGoogle Scholar
  10. 10.
    Hirota S, Isozaki K, Moriyama Y, et al. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science 1998; 279: 577–80.CrossRefPubMedGoogle Scholar
  11. 11.
    Ernst SI, Hubbs AE, Przygodzki RM, Emory TS, Sobin LH, O’Leary TJ. KIT mutation portends poor prognosis in gastrointestinal stromal/smooth muscle tumors. Lab Invest 1998; 78: 1633–6.PubMedGoogle Scholar
  12. 12.
    Seidal T, Edvardsson H. Expression of c-kit (CD117) and Ki67 provides information about the possible cell of origin and clinical course of gastrointestinal stromal tumors. Histopathology 1999; 34: 416–24.PubMedGoogle Scholar
  13. 13.
    Moskaluk CA, Tian Q, Marshall CR, Rumpel CA, Franquemont DW, Frierson HF. Mutations of c-kit JM domain are found in a minority of human gastrointestinal stromal tumors. Oncogene 1999; 18: 1897–1902.CrossRefPubMedGoogle Scholar
  14. 14.
    Lasota J, Jasinski M, Sarlomo-Rikala M, Miettinen M. Mutations in exon 11 of c-kit occur preferentially in malignant versus benign gastrointestinal stromal tumors and do not occur in leiomyomas or leiomyosarcomas. Am J Pathol 1999; 154: 53–60.PubMedGoogle Scholar
  15. 15.
    Taniguchi M, Nishida T, Hirota S, et al. Effect of c-kit mutation on prognosis of gastrointestinal stromal tumors. Cancer Res 1999; 59: 4297–4300.PubMedGoogle Scholar
  16. 16.
    McGrath PC, Neifeld JP, Lawrence W, Kay S, Horsley JS, Parker GA. Gastrointestinal sarcomas. Analysis of prognostic factors. Ann Surg 1987; 206: 706–10.PubMedGoogle Scholar
  17. 17.
    Ng EH, Pollock RE, Munsell MF, Atkinson EN, Romsdahl MM. Prognostic factors influencing survival in gastrointestinal leiomyosarcomas. Implications for surgical management and staging. Ann Surg 1992; 215: 68–77.PubMedCrossRefGoogle Scholar
  18. 18.
    Ng EH, Pollock RE, Romsdahl MM. Prognostic implications of patterns of failure for gastrointestinal leiomyosarcomas. Cancer 1992; 69: 1334–41.PubMedGoogle Scholar
  19. 19.
    Haque S, Dean PJ. Stromal neoplasms of the rectum and anal canal. Hum Pathol 1992; 23: 762–7.PubMedGoogle Scholar
  20. 20.
    Hill MA, Mera R, Levine EA. Leiomyosarcoma. A 45-year review at Charity Hospital, New Orleans. Am Surg 1998; 64: 53–61.PubMedGoogle Scholar
  21. 21.
    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: 82–7.PubMedGoogle Scholar
  22. 22.
    Appelman HD. Smooth muscle tumors of the gastrointestinal tract. What we know now that Stout didn’t know. Am J Surg Pathol 1986; 10 (Suppl 1): 83–99.PubMedGoogle Scholar
  23. 23.
    Ludwig DJ, Traverso LW. Gut stromal tumors and their clinical behavior. Am J Surg 1997; 173: 390–4.PubMedGoogle Scholar
  24. 24.
    Tazawa K, Tsukada K, Makuuchi H, Tsutsumi Y. An immunohistochemical and clinicopathological study of gastrointestinal stromal tumors. Pathol Int 1999; 49: 786–98.PubMedGoogle Scholar
  25. 25.
    Brainard JA, Goldblum JR. Stromal tumors of the jejunum and ileum. A clinicopathologic study of 39 cases. Am J Surg Pathol 1997; 21: 407–16.PubMedGoogle Scholar
  26. 26.
    Tworek JA, Appelman HD, Singleton TP, Greenson JK. Stromal tumors of the jejunum and ileum. Mod Pathol 1997; 10: 200–209.PubMedGoogle Scholar
  27. 27.
    Chang MS, Choe G, Kim WH, Kim YI. Small intestinal stromal tumors: A clinicopathologic study of 31 tumors. Pathol Int 1998; 48: 341–7.CrossRefPubMedGoogle Scholar
  28. 28.
    Horowitz J, Spellman JE, Driscoll DL, Velez AF, Karakousis CP. An institutional review of sarcomas of the large and small intestine. J Am Coll Surg 1995; 180: 465–71.PubMedGoogle Scholar
  29. 29.
    Hermanek P, Henson DE, Hutter RVP, Sobin LH. UICC TMN Supplement 1993: A Commentary on Uniform Use. Berlin: Springer-Verlag, 1993.Google Scholar
  30. 30.
    Kaplan EL, Meier P. Nonparametric estimation from incomplete observations. J Am Stat Assoc 1956; 153: 457–86.Google Scholar
  31. 31.
    Elandt-Johnson RC, Johnson NL. Survival Models and Data Analysis. New York: John Wiley and Sons, 1980.Google Scholar
  32. 32.
    Cox DR, Oakes D. Analysis of Survival Data. London: Chapman and Hall, 1984.Google Scholar
  33. 33.
    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: 51–8.CrossRefPubMedGoogle Scholar
  34. 34.
    Chou FF, Eng HL, Sheen-Chen SM. Smooth muscle tumors of the gastrointestinal tract: Analysis of prognostic factors. Surgery 1996; 119: 171–7.PubMedGoogle Scholar
  35. 35.
    Evans HL. Smooth muscle tumors of the gastrointestinal tract. A study of 56 cases followed for a minimum of 10 years. Cancer 1985; 56: 2242–50.PubMedGoogle Scholar
  36. 36.
    Dougherty MJ, Compton C, Talbert M, Wood WC. Sarcomas of the gastrointestinal tract. Separation into favorable and unfavorable prognostic groups by mitotic count. Ann Surg 1991; 214: 569–74.PubMedGoogle Scholar
  37. 37.
    Rudolph P, Gloeckner K, Parwaresch R, Harms D, Schmidt D. Immunophenotype, proliferation DNA. ploidy, and biological behavior of gastrointestinal stromal tumors: A multivariable clinicopathologic study. Hum Pathol 1998; 29: 791–800.PubMedGoogle Scholar
  38. 38.
    Conlon KC, Casper ES, Brennan MF. Primary gastrointestinal sarcomas: analysis of prognostic variables. Ann Surg Oncol 1995; 2: 26–31.PubMedGoogle Scholar
  39. 39.
    Ueyama T, Guo KJ, Hashimoto H, Daimaru Y, Enjoji M. A clinicopathologic and immunohistochemical study of gastrointestinal stromal tumors. Cancer 1992; 69: 947–55.PubMedGoogle Scholar
  40. 40.
    Crosby J, Catton C, Davis A, O’Sullivan B, Couture J, Swallow C. Malignant GI stromal tumors (M-GIST): 52 cases from a prospective database. Can J Surg (suppl) 1999;42:15.Google Scholar
  41. 41.
    Lev D, Kariv J, Merhav H, et al. Gastrointestinal stromal sarcomas. Br J Surg 1999; 86: 545-49.PubMedGoogle Scholar
  42. 42.
    Appelman HD, Helwig EB. Gastric epithelioid leiomyoma and leiomyosarcoma (leiomyoblastoma). Cancer 1976; 38: 708–28.Google Scholar
  43. 43.
    Miller KA, Rubnitz ME, Roth SI. Late recurrence (33 years) of a gastric epithelioid stromal tumor (leiomyoblastoma) with low malignant potential. Arch Pathol Lab Med 1988; 112: 86–90.PubMedGoogle Scholar
  44. 44.
    Van Steenbergen W, Kojima T, Geboes K, et al. Gastric leiomyoblastoma with metastases to the liver. A 36 year follow-up study. Gastroenterology 1985; 89: 875–81.PubMedGoogle Scholar
  45. 45.
    Mudan SS, Conlon KC, Woodruff JM, Lewis JJ, Brennan MF. Salvage surgery for patients with recurrent gastrointestinal sarcoma. Prognostic factors to guide patient selection. Cancer 2000; 88: 66–74.CrossRefPubMedGoogle Scholar
  46. 46.
    McGrath PC, Sloan DA, Kenady DE. Adjuvant therapy of soft-tissue sarcomas. Clin Plast Surg 1995; 22: 21–9.PubMedGoogle Scholar
  47. 47.
    Shiu MH, Farr GH, Papachristou DN, Hajdu SI. Myosarcomas of the stomach: natural history, prognostic factors and management. Cancer 1982; 49: 177–87.PubMedGoogle Scholar
  48. 48.
    Blair SC, Zalupski MM, Baker LH. Ifosfamide and etoposide in the treatment of advanced soft tissue sarcomas. Am J Clin Oncol 1994; 17: 480–4.PubMedGoogle Scholar
  49. 49.
    Eilber FC, Rosen G, Forscher C, Nelson SD, Dorey FJ, Eilber FR. Surgical resection and intraperitoneal chemotherapy for recurrent abdominal sarcomas. Ann Surg Oncol 1999; 6: 645–50.PubMedGoogle Scholar

Copyright information

© The Society of Surgical Oncology, Inc. 2001

Authors and Affiliations

  • Jacqueline A. Crosby
    • 1
    • 6
  • Charles N. Catton
    • 2
    • 6
  • Aileen Davis
    • 3
  • Jean Couture
    • 1
    • 4
    • 6
  • Brian O’Sullivan
    • 2
    • 6
  • Rita Kandel
    • 5
    • 6
  • Carol J. Swallow
    • 1
    • 3
    • 6
    • 7
  1. 1.Department of Surgical OncologyPrincess Margaret HospitalTorontoCanada
  2. 2.Department of Radiation OncologyPrincess Margaret HospitalTorontoCanada
  3. 3.Musculoskeletal Oncology UnitMount Sinai Hospital and the University of TorontoTorontoCanada
  4. 4.Department of SurgeryMount Sinai HospitalTorontoCanada
  5. 5.Department of Pathology and Laboratory MedicineThe University of Toronto Sarcoma Group, Mount Sinai HospitalTorontoCanada
  6. 6.The University of Toronto Sarcoma GroupTorontoCanada
  7. 7.Mount Sinai HospitalTorontoCanada

Personalised recommendations