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Gastrointestinal Stromal Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - General Information About Gastrointestinal Stromal Tumors Treatment

Table 1. Risk Stratification of Primary GIST by Mitotic Index, Tumor Size, and Tumor Locationa continued...

In the retrospective study of 200 patients cited in Table 1 above, 7% had isolated local recurrence and 47% had metastasis.[11] The site of relapse for GIST is usually intra-abdominal, involving the peritoneum, the liver, or both; true local recurrences are uncommon, and typically there is widespread intraperitoneal recurrence that may not be detectable by imaging techniques.[27] The median disease-specific survival of patients with metastatic GIST (N = 94) was 19 months.[11] In one retrospective study involving 119 patients with metastatic GIST, it was found that once a GIST becomes metastatic, kinase genotype did not factor into overall survival.[28]

The median time to recurrence for patients on imatinib is 2 years.[27]

Follow-up

The most appropriate tests and frequency of testing for metastatic or recurrent disease in patients who have undergone GIST resection are ill-defined, since the impact of follow-up strategies on clinical outcomes is not known. Follow-up recommendations are, therefore, based upon expert opinion and clinical judgment taking into account tumor site, size, and mitotic index. For surgically treated patients with localized disease, routine follow-up schedules may differ across institutions and may depend on the risk status of the tumor.[18] Abdominal/pelvic CT may be performed every 3 to 6 months, but very low-risk lesions may not need routine follow-up testing.[18]

CT or 18FDG-PET are used to monitor therapeutic effects in patients receiving systemic therapy for unresectable, metastatic, or recurrent disease.[27] 18FDG-PET may also be helpful in detecting resistance to TKI. If 18FDG-PET is used to monitor therapy with a TKI, a baseline FDG-PET is often performed before kinase inhibitor administration. Because 18FDG-PET imaging may detect the activity of imatinib in GIST much earlier than CT imaging, imaging of GIST with 18FDG-PET may represent a useful diagnostic modality for the very early assessment of response to imatinib therapy; a decrease in tumor avidity for 18FDG may be detected as early as 24 hours after a single dose of imatinib.[12]

Related Summary

(Refer to the Abdominal Cancers section in the PDQ summary on Unusual Cancers of Childhood Treatment for information on gastrointestinal stromal tumors in children.)

References:

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  2. Corless CL, Heinrich MC: Molecular pathobiology of gastrointestinal stromal sarcomas. Annu Rev Pathol 3: 557-86, 2008.
  3. 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 100 (1): 162-8, 2005.
  4. Kawanowa K, Sakuma Y, Sakurai S, et al.: High incidence of microscopic gastrointestinal stromal tumors in the stomach. Hum Pathol 37 (12): 1527-35, 2006.
  5. Agaimy A, Wünsch PH, Hofstaedter F, et al.: Minute gastric sclerosing stromal tumors (GIST tumorlets) are common in adults and frequently show c-KIT mutations. Am J Surg Pathol 31 (1): 113-20, 2007.
  6. Nowain A, Bhakta H, Pais S, et al.: Gastrointestinal stromal tumors: clinical profile, pathogenesis, treatment strategies and prognosis. J Gastroenterol Hepatol 20 (6): 818-24, 2005.
  7. Gastrointestinal stromal tumor. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 175-80.
  8. Corless CL, Fletcher JA, Heinrich MC: Biology of gastrointestinal stromal tumors. J Clin Oncol 22 (18): 3813-25, 2004.
  9. Joensuu H: Gastrointestinal stromal tumor (GIST). Ann Oncol 17 (Suppl 10): x280-6, 2006.
  10. Miettinen M, Lasota J: Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis. Arch Pathol Lab Med 130 (10): 1466-78, 2006.
  11. DeMatteo RP, Lewis JJ, Leung D, et al.: Two hundred gastrointestinal stromal tumors: recurrence patterns and prognostic factors for survival. Ann Surg 231 (1): 51-8, 2000.
  12. Demetri GD: Gastrointestinal stromal tumor. In: DeVita VT Jr, Lawrence TS, Rosenberg SA: Cancer: Principles and Practice of Oncology. 9th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2011, pp 1060-73.
  13. Nickl NJ: Gastrointestinal stromal tumors: new progress, new questions. Curr Opin Gastroenterol 20 (5): 482-7, 2004.
  14. Vander Noot MR 3rd, Eloubeidi MA, Chen VK, et al.: Diagnosis of gastrointestinal tract lesions by endoscopic ultrasound-guided fine-needle aspiration biopsy. Cancer 102 (3): 157-63, 2004.
  15. Hirota S, Isozaki K, Moriyama Y, et al.: Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science 279 (5350): 577-80, 1998.
  16. Kindblom LG, Remotti HE, Aldenborg F, et al.: Gastrointestinal pacemaker cell tumor (GIPACT): gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal. Am J Pathol 152 (5): 1259-69, 1998.
  17. Antonescu CR: Targeted therapy of cancer: new roles for pathologists in identifying GISTs and other sarcomas. Mod Pathol 21 (Suppl 2): S31-6, 2008.
  18. Casali PG, Jost L, Reichardt P, et al.: Gastrointestinal stromal tumors: ESMO clinical recommendations for diagnosis, treatment and follow-up. Ann Oncol 19 (Suppl 2): ii35-8, 2008.
  19. Heinrich MC, Corless CL, Duensing A, et al.: PDGFRA activating mutations in gastrointestinal stromal tumors. Science 299 (5607): 708-10, 2003.
  20. Andersson J, Sihto H, Meis-Kindblom JM, et al.: NF1-associated gastrointestinal stromal tumors have unique clinical, phenotypic, and genotypic characteristics. Am J Surg Pathol 29 (9): 1170-6, 2005.
  21. Agaimy A, Pelz AF, Corless CL, et al.: Epithelioid gastric stromal tumours of the antrum in young females with the Carney triad: a report of three new cases with mutational analysis and comparative genomic hybridization. Oncol Rep 18 (1): 9-15, 2007.
  22. Carney JA: Gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma (Carney Triad): natural history, adrenocortical component, and possible familial occurrence. Mayo Clin Proc 74 (6): 543-52, 1999.
  23. Miettinen M, Sobin LH, Lasota J: Gastrointestinal stromal tumors of the stomach: a clinicopathologic, immunohistochemical, and molecular genetic study of 1765 cases with long-term follow-up. Am J Surg Pathol 29 (1): 52-68, 2005.
  24. Miettinen M, Makhlouf H, Sobin LH, et al.: Gastrointestinal stromal tumors of the jejunum and ileum: a clinicopathologic, immunohistochemical, and molecular genetic study of 906 cases before imatinib with long-term follow-up. Am J Surg Pathol 30 (4): 477-89, 2006.
  25. Miettinen M, Kopczynski J, Makhlouf HR, et al.: Gastrointestinal stromal tumors, intramural leiomyomas, and leiomyosarcomas in the duodenum: a clinicopathologic, immunohistochemical, and molecular genetic study of 167 cases. Am J Surg Pathol 27 (5): 625-41, 2003.
  26. Miettinen M, Furlong M, Sarlomo-Rikala M, et al.: Gastrointestinal stromal tumors, intramural leiomyomas, and leiomyosarcomas in the rectum and anus: a clinicopathologic, immunohistochemical, and molecular genetic study of 144 cases. Am J Surg Pathol 25 (9): 1121-33, 2001.
  27. Demetri GD, Benjamin RS, Blanke CD, 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 5 (Suppl 2): S1-29; quiz S30, 2007.
  28. Gold JS, van der Zwan SM, Gönen M, et al.: Outcome of metastatic GIST in the era before tyrosine kinase inhibitors. Ann Surg Oncol 14 (1): 134-42, 2007.
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Last Updated: February 25, 2014
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