Details
Category: Volume 06 Issue 06 June 2018
Hits: 1207

Title: An Unusual presentation of Gastric Gastro intestinal Stromal Tumour: A Case Report

Authors: Waqar Ahmed Ansari, Priyanka Saha, Rohan Khairatkar, Madhukar Gupta, Dnyaneshwar Raut, Metsivi Iralu

 DOI:  https://dx.doi.org/10.18535/jmscr/v6i6.128

Abstract

Gastro-intestinal stromal tumours (GIST) are uncommon malignancies of the gastro-intestinal tract and can occur in any part of the alimentary canal , from esophagus to anus. The most common sites of presentation are stomach, small bowel and rectum.95% of GIST are positive for KIT (CD117) and Platelet Derived Growth factor Receptor Alpha (PDGFRA). Treatment of choice is complete surgical excision with negative margins (R0 resection).

A 68 years old male presented with slowly progressive abdominal lump in the right iliac fossa. CECT abdomen suggestive of hypervascular heterogenous mass arising from pelvis with low central attenuation with no distant metastasis. Patient was taken up for exploratory laparotomy and intra operatively it was found to be a huge exophytic, well circumscribed tumour arising from the lesser curvature of stomach , complete surgical resection was done. Microscopically diagnosis of gastric GIST of spindle cell morphology was found which was strongly positive for DOG1/CD34 and immunonegative for CD117. Medical oncology reference was taken on follow up and patient was started on imatinib therapy.

This case report sites a rare presentation of gastric GIST in right iliac fossa with inconclusive CT reports. So a high index of suspicion must be kept in mind for diagnosing such rare tumours.

Keywords: Gastro intestinal stromal tumours, GIST, abdominal lump, CD117, DOG 1, imatinib,

References

  1. H.F., L. (2007). Gastro-intestinal stromal tumours: A review of current management options. Hong Kong Medical Journal, 13(1), 61–65.
  2. Heinrich MC,  Corless CL,  Duensing A, et al. PDGFRA activating mutations in gastrointestinal stromal tumours, Science, 2003, vol. 299 (pg. 708-710)
  3. Nishida T, Hirota S, Taniguchi M, Hashimoto K, Isozaki K, Nakamura H, et al. Familial gastrointestinal stromal tumours with germline mutation of the KIT gene. Nat Genet. 1998;19:323–4.
  4. Corless CL, Fletcher JA, Heinrich MC. Biology of gastrointestinal stromal tumors. J Clin Oncol. 2004;22(18):3813–3825. [PubMed]
  5. N. Wente, M.W. Buchler, J. Weitz Gastrointestinal stromal tumors (GIST). Surgical therapy Chirurg, 79 (2008), pp. 638-643
  6. A. Sexton, R.A. Pierce, V.J. Halpin, et al Laparoscopic gastric resection for gastrointestinal stromal tumors Surg Endosc, 22 (2008), pp. 2583-2587
  7. http://www.ncbi.nlm.nih.gov/pubmed/17193820
  8. Kindblom LG, Remotti HE, Aldenborg F, Meis-Kindblom JM. Gastrointestinal stromal tumours show phenotypic characteristics of the interstitial cells of Cajal. Am J Pathol. 1998;152:1259–1269
  9. Duensing, A. et al. Mechanisms of oncogenic KIT signal transduction in primary gastrointestinal stromal tumors (GISTs). Oncogene23, 3999–4006, 10.1038/sj.onc.1207525 1207525 (2004).
  10. Hirota, S. et al. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science 279, 577–580 (1998)
  11. Wozniak, A. et al. Prognostic value of KIT/PDGFRA mutations in gastrointestinal stromal tumours (GIST): Polish Clinical GIST Registry experience. Ann Oncol23, 353–360, 10.1093/annonc/mdr127 mdr127 (2012).
  12. Choi H, Charnsangavej C, Faria SC, et al. Correlation of computed tomography and positron emission tomography in patients with metastatic gastrointestinal stromal tumor treated at a single institution with imatinib mesylate: proposal of new computed tomography response criteria. J Clin Oncol 2007;25:1753–9. [PubMed]
  13. Bamboat ZM, DeMatteo RP. Updates on the management of gastrointestinal stromal tumors (GIST). Surg Oncol Clin N Am 2012;21:301–16. [PMC free article] [PubMed]
  14. .Joensuu H, Fletcher C, Dimitrijevic S, Silberman S, Roberts P, Demetri G. Management of malignant gastrointestinal stromal tumours. Lancet Oncol. 2002;3 (11):655–664. [PubMed]
  15. Miettinen M, Virolainen M, Maarit Sarlomo R. Gastrointestinal stromal tumour of the stomach: a clinicopathologic, immunohisto-chemical and molecular genetic study of 1765 cases with long-term follow-up. Am J Surg Pathol. 2005;29:52–68. doi: 10.1097/01.pas.0000146010.92933.de. [PubMed] [Cross Ref]
  16. Cegarra-Navarro MF, de la Calle MA, Girela-Baena E, García-Santos JM, Lloret-Estañ F, de Andrés EP. Ruptured gastrointestinal stromal tumors: radiologic findings in six cases. Abdom Imaging. 2005;30:535–542. doi: 10.1007/s00261-005-0308-6. [PubMed] [Cross Ref]
  17. Ma, Y. Y. et al. Involvement of c-KIT mutation in the development of gastrointestinal stromal tumors through proliferation promotion and apoptosis inhibition. Onco Targets Ther7, 637–643, 10.2147/OTT.S60458 ott-7-637 (2014)
  18. De Silva MVC, Reid R. Gastrointestinal Stromal Tumours (GIST): C-kit Mutations, CD 117 Expression, Differtial Diagnosis and Targeted Cancer Therapy with Imatinib. Pathol Oncol Res. 2003;9:13–19. [PubMed]
  19. Chabot, B., Stephenson, D. A., Chapman, V. M., Besmer, P. & Bernstein, A. The proto-oncogene c-kit encoding a transmembrane tyrosine kinase receptor maps to the mouse W locus. Nature 335, 88–89, 10.1038/335088a0 (1988).
  20. Emile, J. F. et al. Clinicopathologic, phenotypic, and genotypic characteristics of gastrointestinal mesenchymal tumors. Clin Gastroenterol Hepatol2, 597–605, S1542356504002435 (2004).
  21. International union against cancer (UICC) Sobin LH, Wittekind Ch, editors. 7th ed. New York: Wiley; 2010. TNM classification of malignant tumours.
  22. Fletcher CD, Berman JJ, Corless C, Gorstein F, Lasota J, Longley BJ, Miettinen M, O'Leary TJ, Remotti H, Rubin BP, Shmookler B, Sobin LH, Weiss SW. Diagnosis of gastrointestinal stromal tumors: A consensus approach. Hum Pathol. 2002;33:459–465. [PubMed]
  23. International union against cancer (UICC)Sobin LH, Wittekind Ch, editors. 7th ed. New York: Wiley; 2010. TNM classification of malignant tumours.
  24. Demetri GD, von Mehren M, Blanke CD, et al. Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors. N Engl J Med. 2002;347(7):472–480. [PubMed]
  25. Dematteo RP, Ballman KV, Antonescu CR, et al. American College of Surgeons Oncology Group (ACOSOG) Intergroup Adjuvant GIST Study Team Adjuvant imatinib mesylate after resection of localised, primary gastrointestinal stromal tumour: a randomised, double-blind, placebo controlled trial. Lancet. 2009;373 (9669):1097–1104. [PMC free article] [PubMed]
  26. Nilsson B, Sjölund K, Kindblom LG, et al. Adjuvant imatinib treatment improves recurrence-free survival in patients with high-risk gastrointestinal stromal tumours (GIST) Br J Cancer. 2007;96(11):1656–1658. [PMC free article] [PubMed]
  27. Demetri GD, von Mehren M, Blanke CD, et al. Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors. N Engl J Med.

Corresponding Author

Priyanka Saha

Email: This email address is being protected from spambots. You need JavaScript enabled to view it.