Gastrointestinal stromal tumor of the ampulla of Vater: A case report and literature review

INTRODUCTION: Gastrointestinal stromal tumors (GISTs) usually develop in the stomach and small intestine and only rarely occur in the ampulla of Vater (AV). CASE PRESENTATION: We report a case of a GIST of the AV. A 21-year-old, previously healthy woman presented with a three-month history of epigastric pain, jaundice and weight loss. The diagnosis of a tumor in the second part of the duodenum was made by the computed tomography. The patient underwent pancreatoduodenectomy, the operative specimen revealed an intermediate risk group of GIST and thus, the patient didn't take adjuvant therapy. DISCUSSION: GIST rarely develops in the duodenal ampulla region and it has no specific symptomatology. Radiological investigations play an important role in the diagnosis of Stromal Tumor of the Ampulla of Vater (STAV) mainly computed tomography because STAV has a large size in the majority of cases contrary to adenocarcinoma. CONCLUSION: The aim of this study is to report a new case of STAV combined with a systematic review of reported cases published in peer-reviewed journals.