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Abstract

Background: Extragastrointestinal stromal tumors are unusual, with those arising in the pancreas even more so. Because extragastrointestinal stroma tumors involving the pancreas may present nonspecifically as an upper gastrointestinal bleed, it is important to adequately work up the patient to rule out malignancy to optimize the outcome for the patient. While surgery with complete resection of the tumor is the treatment of choice, it is important to consider the role of tumor markers in targeted therapy for a nonresectable disease or nonsurgical patients. Case presentation:We describe a 53-year old man with a history of neurofibromas and irritable bowel syndrome who presented with abdominal pain and persistent iron deficiency anemia. He was found to have an extragastrointestinal stromal tumor of the pancreas, which stained positive for CD117 and DOG1. The treatment plan for this patient is targeted therapy with Imatinib, a tyrosine kinase inhibitor effective in the treatment of CD117+ cancer cells, followed by possible surgical resection. Conclusions:The pancreatic extragastrointestinal stromal tumor is a rare malignancy that can present with acute, nonspecific findings of a gastrointestinal bleed including anemia, fatigue, and melanotic bowel movements. With adequate history and physical examination, we can appropriately work up a patient with this rare disease and develop an appropriate treatment plan for the patient.