Pancreatic GIST with pancreas divisum: A new entity.

ABSTRACT

INTRODUCTION: Gastrointestinal stromal tumours (GISTs) are uncommon intra-abdominal tumours. These tumours tend to arise with a higher frequency in the stomach and the small bowel. In fewer than 5% of cases, they originate primarily from extra-gastrointestinal tumours (EGISTs). Gastrointestinal stromal tumour of the pancreas is very rare. Only few cases have been published. We report the first case of stromal tumour of the pancreas with concomitant pancreas divisum. PRESENTATION OF CASE A 39-year-old male who presented with constipation and abdominal pain. A computerized tomography demonstrated a 9cm pancreatic mass, without liver lesions. A Whipple procedure with segmental colectomy was performed with success. After 24months follow up, the patient is doing well and disease-free. DISCUSSION: Mesenchymal tumours of the pancreas are extremely rare, accounting for less than 1% of all pancreatic tumours. The endoscopic ultrasound is helpful for diagnosis. Surgical resection with negative pathologic margins remains the treatment of choice. CONCLUSION: in our knowledge, this is the first case of pancreatic GIST with pancreas divisum. Although pancreatic GISTs are uncommon tumours, they must be considered in the differential diagnosis of solid pancreatic lesions. Even though the tumour can be evaluated as high risk, treatment must be aggressive in order to improve survival rate.