Abstract

Gastrointestinal stromal tumors (GISTs) are rare mesenchymal tumors arising from the gastrointestinal tract. Stromal tumors arising outside the gastrointestinal tract are called extra-gastrointestinal stromal tumors, usually found in omentum, mesentery, retroperitoneum and gallbladder. We report a case of 31 years old male with 3-month history of painless jaundice and undocumented weight loss. Computerized tomography scan showed a well-defined, rounded enhancing lesion in the uncinate process of pancreas measuring 53x49mm compressing the distal common bile duct. Percutaneous biopsy confirmed gastrointestinal stromal tumor. He underwent Whipple’s procedure followed by adjuvant imatinib due to high-risk features. He remained well for a long time and developed oligo-metastasis in liver 5 years after resection while he was still on imatinib. The patient underwent hepatic resection and remains well one year after the second resection on adjuvant treatment. Pancreatic extragastrointestinal stromal tumor involving uncinate process is a rare disease entity. First line of management is negative margin resection followed by adjuvant therapy with imatinib for high-risk tumors. In case of resectable liver metastasis, resection of metastasis followed by multimodal therapy seems to prolong the disease free and overall survival.

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