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Gastrointestinal stromal tumors

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Opinion statement

The nonepithelial, nonlymphoid tumors of the gastrointestinal tract are heterogeneous in terms of clinical presentation, behavior, pathology, and genetic features. Concepts regarding these tumors have changed rapidly over the past decade as nomenclature has evolved. Many of these tumors have no muscle differentiation, and designations such as leiomyoma or leiomyosarcoma are inappropriate for many of these neoplasms. With an improved understanding of the biology of these tumors, gastrointestinal stromal tumor (GIST) is used as a specific term for tumors of the gastrointestinal tract that lack markers of myogenic differentiation, but stain positive for vimentin, and express CD34 and CD117, the product of the c-kit oncogene. Both benign and malignant types are recognized. In addition to myogenic tumors and GIST, gastrointestinal autonomic nerve tumors (GANT) are also recognized.

Complete en bloc surgical resection, when possible, is the cornerstone of therapy. Metastasis tends to occur to the liver and within the peritoneal cavity, especially in patients whose tumors have ruptured spontaneously or been violated by the surgeon. Incomplete surgical resection and metastatic disease indicate a dismal prognosis in the majority of patients.

Recurrent or metastatic disease is often resected, but this has an uncertain impact on outcome. Operation may palliate patients with intestinal obstruction or other symptoms. For patients with unresectable disease, the results with systemic chemotherapy have been dismal. Treatment with doxorubicin/ifosfamide combinations is of dubious value. Hepatic arterial embolization, with and without intra-arterial chemotherapy, results in regression of liver metastases in selected patients. Regression has also been seen using intrahepatic arterial infusion of doxorubicin without embolization. The impact of such treatment on outcome, however, is poorly studied. Aggressive surgical resection of peritoneal metastases with intraperitoneal chemotherapy has been advocated, but requires formal study in large trials.

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References and Recommended Reading

  1. Elman HD: Smooth muscle tumors of the gastrointestinal tract: what we know that Stout didn’t know. Am J Surg Pathol 1986, 10:83–99.

    Google Scholar 

  2. Grath PC, Neifeld JP, Lawrence Jr W, et al.: Gastrointestinal sarcomas. Ann Surg 1987, 206:706–710.

    Article  Google Scholar 

  3. Lauwers GY, Erlandson RA, Casper ES, et al.: Gastrointestinal autonomic nerve tumors: a clinicopathological, immunohistochemical, and ultrastructural study of 12 cases. Am J Surg Pathol 1993, 17:887–897.

    Article  PubMed  CAS  Google Scholar 

  4. Sircar K, Hewlett BR, Huizinga JD, et al.: Interstitial cells of Cajal as precursors of gastrointestinal stromal tumors. Am J Surg Pathol 1999, 23:377–389. The authors demonstrate that the interstitial cells of Cajal are the only vimentin+, CD34+, Kit+ cells in the gut, and that GIST shows differentiation along similar lines. The article has an excellent discussion of the immunopathology of these tumors.

    Article  PubMed  CAS  Google Scholar 

  5. Miettinen M, Sarlomo-Rikala M, Lasota J: Gastrointestinal stromal tumors: recent advances in understanding of their biology. Hum Pathol 1999, 30:1213–1220. An up-to-date review of GIST as a specific entity, this article provides both pathologic and clinical data.

    Article  PubMed  CAS  Google Scholar 

  6. Miettinen M, Sarlomo-Rikala M, Lasota J: Gastrointestinal stromal tumours. Ann Chir Gynaecol 1998, 87:278–281. This review provides a comprehensive discussion of the entity by authors who have studied this tumor extensively.

    PubMed  CAS  Google Scholar 

  7. Vliagoftis H, Worobec AS, Metcalfe DD: The protooncogene c-kit and c-kit ligand in human disease. J Allerg Clin Immunol 1997, 100:435–440.

    Article  CAS  Google Scholar 

  8. Erlandson RA, Klimstra DS, Woodruff JM: Subclassification of gastrointestinal stromal tumors based on evaluation by electron microscopy and immunohistochemistry. Ultrastruct Pathol 1996, 20:373–393.

    PubMed  CAS  Google Scholar 

  9. Miettinen M, Monihan JM, Sarlomo-Rikala M, et al.: Gastrointestinal stromal tumors/smooth muscle tumors (GISTs) primary in the omentum and mesentery: clinicopathologic and immunohistochemical study of 26 cases. Am J Surg Pathol 1999, 23:1109–1118. Convincing data are presented to demonstrate that GIST occurs outside the gastrointestinal tract.

    Article  PubMed  CAS  Google Scholar 

  10. el-Rifai W, Sarlomo-Rikala M, Andersson LC, et al.: DNA copy number changes in gastrointestinal stromal tumors: a distinct genetic entity. Ann Chir Gynaecol 1998, 87:287–290.

    PubMed  CAS  Google Scholar 

  11. Conlon KC, Casper ES, Brennan MF: Primary gastrointestinal sarcomas: analysis of prognostic variables. Ann Surg Oncol 1995, 2:26–31.

    Article  PubMed  CAS  Google Scholar 

  12. Emory TS, Sobin LH, Lukes L, et al.: Prognosis of gastrointestinal smooth-muscle (stromal) tumors: dependence on anatomic site. Am J Surg Pathol 1999, 23:82–87. This is the largest collection of cases of gastrointestinal stromal tumors in the literature, reviewing the Armed Forces institute of Pathology experience with 1004 patients. The prognostic importance of site is demonstrated. Unfortunately these authors fail to analyze the results by specific entity.

    Article  PubMed  CAS  Google Scholar 

  13. Ng EH, Pollock RE, Romsdahl MM: Prognostic implications of patterns of failure for gastrointestinal leiomyosarcomas. Cancer 1992, 69:1334–1341.

    Article  PubMed  CAS  Google Scholar 

  14. Buckley JA, Fishman EK: CT evaluation of small bowel neoplasms: spectrum of disease. Radiographics 1998, 18:379–392.

    PubMed  CAS  Google Scholar 

  15. Case records of the Massachusetts General Hospital: weekly clinicopathological exercises: case 15-1993: 58-year-old man with recurrent lower gastrointestinal bleeding for over two years. N Engl J Med 1993, 328:1107–1114.

  16. Chak A, Canto MI, Rosch T, et al.: Endosonographic differentiation of benign and malignant stromal cell tumors. Gastrointest Endosc 1997, 45:468–473.

    Article  PubMed  CAS  Google Scholar 

  17. Fuller CE, Williams GT: Gastrointestinal manifestations of type 1 neurofibromatosis (von Recklinghausen’s disease). Histopathology 1991, 19:1–11.

    Article  PubMed  CAS  Google Scholar 

  18. Carney JA: Gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma (Carney Triad): natural history, adrenocortical component, and possible familial occurrence. Mayo Clin Proc 1999, 74:543–552.

    Article  PubMed  CAS  Google Scholar 

  19. Nishida T, Hirota S, Taniguchi M, et al.: Familial gastrointestinal stromal tumours with germline mutation of the KIT gene [letter]. Nat Genet 1998, 19:323–324.

    Article  PubMed  CAS  Google Scholar 

  20. 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.

    PubMed  CAS  Google Scholar 

  21. Ernst SI, Hubbs AE, Przygodzki RM, et al.: KIT mutation portends poor prognosis in gastrointestinal stromal/smooth muscle tumors. Lab Invest 1998, 78:1633–1636.

    PubMed  CAS  Google Scholar 

  22. Berthet B, Sugarbaker TA, Chang D, Sugarbaker PH: Quantitative methodologies for selection of patients with recurrent abdominopelvic sarcoma for treatment. Eur J Cancer 1999, 35:413–419.

    Article  PubMed  CAS  Google Scholar 

  23. Eilber FC, Eilber FR, Rosen G, Forscher C: Surgical resection and adjuvant intraperitoneal chemotherapy for recurrent abdominal sarcomas. Presented at the 3rd Annual Connective Tissue Oncology Society Scientific Meeting. Milan, Italy, November, 1997.

    Google Scholar 

  24. Jaques DP, Coit DG, Casper ES, Brennan MF: Hepatic metastases from soft-tissue sarcoma. Ann Surg 1995, 221:392–397.

    Article  PubMed  CAS  Google Scholar 

  25. Mavligit GM, Zukwiski AA, Ellis LM, et al.: Gastrointestinal leiomyosarcoma metastatic to the liver. Durable tumor regression by hepatic chemoembolization infusion with cisplatin and vinblastine. Cancer 1995, 75:2083–2088.

    Article  PubMed  CAS  Google Scholar 

  26. Van Glabbeke M, van Oosterom AT, Oosterhuis JW, et al.: Prognostic factors for the outcome of chemotherapy in advanced soft tissue sarcoma: an analysis of 2185 patients treated with anthracycline-containing first-line regimens. European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group Study. J Clin Oncol 1999, 17:150–157.

    PubMed  Google Scholar 

  27. Zalupski M, Metch B, Balcerzak S, et al.: Phase III comparison of doxorubicin and dacarbazine given by bolus versus infusion in patients with soft-tissue sarcomas. Southwest Oncology Group study. J Natl Cancer Inst 1991, 83:926–932.

    Article  PubMed  CAS  Google Scholar 

  28. Edmonson JH, Ryan LM, Blum RH, et al.: Randomized comparison of doxorubicin alone versus ifosfamide plus doxorubicin or mitomycin, doxorubicin, and cisplatin against advanced soft tissue sarcomas. J Clin Oncol 1993, 11:1269–1275.

    PubMed  CAS  Google Scholar 

  29. Antman KH, Ryan L, Elias A, et al.: Response to ifosfamide and mesna: 124 previously treated patients with metastatic or unresectable sarcoma. J Clin Oncol 1989, 7:126–131.

    PubMed  CAS  Google Scholar 

  30. Patel SR, Jenkins J, Papadopoulos NE, et al.: Preliminary results of a two-arm phase 2 trial of gemcitabine (Gem) in patients (Pts) with gastrointestinal leiomyosarcomas (leios) and other soft-tissue sarcomas (STS). Proc ASCO 1999, 18:541a.

    Google Scholar 

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Authors and Affiliations

  1. Chief, Medical Oncology, Memorial Sloan Kettering at Saint Clare’s, 23 Pocono Road, 07834, Denville, NJ, USA

    Ephraim S. Casper MD

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  1. Ephraim S. Casper MD
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Casper, E.S. Gastrointestinal stromal tumors. Curr. Treat. Options in Oncol. 1, 267–273 (2000). https://doi.org/10.1007/s11864-000-0039-4

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  • DOI: https://doi.org/10.1007/s11864-000-0039-4

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