Extragastrointestinal Stromal Tumor Arising in the Pancreas: A Case Report with a Review of the Literature

  • Somanath Padhi Department of Pathology, Nizam’s Institute of Medical Sciences. Panjagutta, Hyderabad, Andhra Pradesh, India
  • Ravikanth Kongara Department of Surgical Gastroenterology, Nizam’s Institute of Medical Sciences. Panjagutta, Hyderabad, Andhra Pradesh, India
  • Shantveer G Uppin Department of Pathology, Nizam’s Institute of Medical Sciences. Panjagutta, Hyderabad, Andhra Pradesh, India
  • Megha S Uppin Department of Pathology, Nizam’s Institute of Medical Sciences. Panjagutta, Hyderabad, Andhra Pradesh, India
  • Aruna K Prayaga Department of Pathology, Nizam’s Institute of Medical Sciences. Panjagutta, Hyderabad, Andhra Pradesh, India
  • Sundaram Challa Department of Pathology, Nizam’s Institute of Medical Sciences. Panjagutta, Hyderabad, Andhra Pradesh, India
  • Bheerappa Nagari Department of Surgical Gastroenterology, Nizam’s Institute of Medical Sciences. Panjagutta, Hyderabad, Andhra Pradesh, India
  • Shastry A Regulagadda Department of Surgical Gastroenterology, Nizam’s Institute of Medical Sciences. Panjagutta, Hyderabad, Andhra Pradesh, India
Keywords: Gastrointestinal Stromal Tumors, Pancreas, Proto-Oncogene Proteins c-kit

Abstract

Context Extragastrointestinal stromal tumors arising in the pancreas are extremely rare. To date, only eight cases have been reported in the literature. Case report A 42-year-old female patient presented with gradually increasing abdominal pain of 6-month duration. Computerized tomography scan of the abdomen demonstrated a solid cystic mass in the body and tail of the pancreas. En-block R0 resection of the mass with distal pancreatectomy, splenectomy and left hemicolectomy was carried out following a radiological diagnosis of a malignant cystic neoplasm of the pancreas. Histopathological and immunohistochemical findings of the lesion were consistent with a gastrointestinal stromal tumor. Conclusion Extragastrointestinal stromal tumor of the pancreas, though rare, should be considered in the differential diagnosis of the more common cystic lesions at this site.

Image: Large lobulated mass in relation to the body and tail of the pancreas.

Downloads

Download data is not yet available.

References

Weiss SW, Goldblum JR. Extra gastrointestinal stromal tumors. In: Enzinger and Weiss's Soft Tissue Tumors; fifth edition. Mosby Elesevier, 2008; p565-79. [ISBN 978-0-323-04628-2]

Ortiz-Hidalgo C, de Leon Bojorge B, Albores-Saavedra J. Stromal tumor of the gallbladder with phenotype of interstitial cells of Cajal: a previously unrecognized neoplasm. Am J Surg Pathol 2000; 24:1420-3. [PMID 11023105]

Neto MR, Machuca TN, Pinho RV, Yuasa LD, Bleggi-Torres LF. Gastrointestinal stromal tumor: report of two unusual cases. Virchows Arch 2004; 444:594-6. [PMID 15118853]

Yamaura K, Kato K, Miyazawa M, Haba Y, Muramatsu A, Miyata K, Koide N. Stromal tumor of the pancreas with expression of c-kit protein: report of a case. J Gastroenterol Hepatol 2004; 19:467-70. [PMID 15012791]

Krska Z, Pesková M, Povýsil C, Horejs J, Sedlácková E, Kudrnová Z. GIST of pancreas. Prague Med Rep 2005; 106:201-8. [PMID 16315768]

Daum O, Klecka J, Ferda J, Treska V, Vanecek T, Sima R, et al. Gastrointestinal stromal tumor of the pancreas:Case report with documentation of KIT gene mutation. Virchows Arch 2005; 446:470-2. [PMID 15756592]

Showalter SL, Lloyd JM, Glassman DT, Berger AC. Extra-gastrointestinal stromal tumor of the pancreas: case report and a review of the literature. Arch Surg 2008; 143:305-8. [PMID 18347279]

Yan B M, Pai R K, Dam J V. Diagnosis of pancreatic gastrointestinal stromal tumor by EUS guided FNA. JOP. J Pancreas (Online) 2008; 9:192-6. [PMID 18326928]

Harindhanavudhi T, Tanawuttiwat T, Pyle J, Silva R. Extra-gastrointestinal stromal tumor presenting as hemorrhagic pancreatic cyst diagnosed by EUS-FNA. JOP. J Pancreas (Online) 2009; 10:189-91. [PMID 19287116]

Trabelsi A, Yacoub-Abid L B, Mtimet A, Ben Abdelkrim S, Hammedi F, Ben Ali A, Mokni M. Gastrointestinal stromal tumor of the pancreas: A case report and review of the literature. North Am J Med Sci 2009; 1:324-6.

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; 13:577-85. [PMID 10824931]

Lecoin L, Gabella G, Le Douarin N. Origin of the c-kit positive interstitial cells in the avian bowel. Development 1996; 122:725-33. [PMID 8631250]

Burns AJ, Herbert TM, Ward SM, Sanders KM. Interstitial cells of Cajal in the guinea-pig gastrointestinal tract as revealed by c-kit immunohistochemistry. Cell Tissue Res 1997; 290:11-20. [PMID 9377631]

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; 28:479-88. [PMID 15087667]

Popescu LM, Hinescu ME, Ionescu N, Ciontea SM, Cretoiu D, Ardelean C. Interstitial cells of Cajal in pancreas. J Cell Mol Med 2005; 9:169-90. [PMID 15784175]

Miettinen M. Are desmoid tumors kits positive? Am J Surg Pathol 2001; 25:549-50. [PMID 11257638]

Large lobulated mass in relation to the body and tail of the pancreas
Published
2010-05-05
How to Cite
PadhiS., KongaraR., UppinS., UppinM., PrayagaA., ChallaS., NagariB., & RegulagaddaS. (2010). Extragastrointestinal Stromal Tumor Arising in the Pancreas: A Case Report with a Review of the Literature. JOP. Journal of the Pancreas, 11(3), 244-248. https://doi.org/10.6092/1590-8577/3821
Section
CASE REPORTS