Extra-gastrointestinal stromal tumor of the pancreas: A case report

INTRODUCTION: Gastrointestinal stromal tumors (GIST) represent the most common mesenchymal tumors of the gastrointestinal tract. In rare cases, these tumors do not develop in the gastrointestinal tract and are defined as extra-gastro-intestinal stromal tumors (EGISTs). Pancreatic EGIST is extremely rare. We reported a case of EGIST of the pancreas in a 53-year-old patient. PRESENTATION OF CASE: A 53-year-old man presented with chronic epigastralgia. An enhanced CT scan showed a solido-cystic tumor of the pancreatic body-tail. The patient underwent a laparotomy. Cytology fine needle aspiration did not find any tumor cells. Enucleation was performed. Histopathology and immunohistochemical examination confirmed the diagnosis of EGIST (CD117 +, Dog-1 +) with a high risk of malignancy. The patient received adjuvant therapy. There was no evidence of disease recurrence after 8 months of follow-up. CLINICAL DISCUSSION: We reported a rare case of a pancreatic EGIST. Enucleation was performed permitting to avoid distal pancreatectomy and thus decreasing morbidity rates. The clinical presentation is not specific and it depends on the location and the size of the tumor. CT scan shows hypervascular tumors with no regional lymph nodes metastasis but is not accurate in assessing diagnosis. Whenever possible, enucleation should be performed. This tumor should be considered in the differential diagnoses of pancreatic neoplasms. CONCLUSION: Pancreatic stromal tumor is extremely rare. There are no specific clinical and radiologic findings. A careful decision should be made after a discussion in a multidisciplinary coordination meeting. Surgical resection is the cornerstone of the treatment. Whenever possible, enucleation is sufficient.