Abstract

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract and usually occur in the stomach and the small intestine. The pancreas is an extremely rare primary site for GISTs and there are 25 reported cases of pancreatic GIST with most being treated with surgical resection. We describe a 52-year-old African-American female who was diagnosed with limited stage small cell carcinoma in November 2009 and treated with concurrent cisplatin/etoposide chemotherapy and radiation. She subsequently achieved complete remission. Two years later she was diagnosed with localized pancreatic GIST by endoscopic ultrasonography guided fine needle aspiration. We treated her with a tyrosine kinase inhibitor (TKI) imatinib 400 mg oral dose daily as she declined surgery. Her disease is stable based on computed tomography imaging scans 40 months after diagnosis without any metastasis. To the best of our knowledge, our case is the second case of localized pancreatic GIST treated with TKI monotherapy.

Highlights

  • Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract and usually occur in the stomach and the small intestine

  • GISTs are group of tumors showing differentiation or derived from the interstitial cells of Cajal which works as the GI pacemaker cells and like GISTs, these cells express both KIT and CD34 [4, 5]

  • Wild-type adult GIST tumors are associated with activation of the succinate dehydrogenase (SDH) complex like cases of GIST associated with Carney triad or Neurofibromatosis 1 [8]

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Summary

Introduction

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract and usually occur in the stomach and the small intestine. The pancreas is an extremely rare primary site for GISTs. The annual incidence of GIST in the United States is 5000–6000/year and they are more common in the males, blacks, and the elderly [1, 2]. The annual incidence of GIST in the United States is 5000–6000/year and they are more common in the males, blacks, and the elderly [1, 2] Workup of these lesions includes morphologic study and immunohistochemical and molecular diagnostic analysis. These neoplasms had been included under a number of diagnostic categories including leiomyoma, leiomyosarcoma, schwannoma, and leiomyoblastoma. Surgery was the only available treatment and this changed in 2001 after discovery of mutational activation of the KIT or PDGFRA genes [3] and the use of targeted therapies

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