Somatostatinoma of the papilla of Vater and duodenal gastrointestinal stromal tumor in a patient with von Recklinghausen's disease

Abstract

A 67-year-old male patient with von Recklinghausen's disease was reffered to our hospital. Abdominal CT showed dilatation of the biliary tract and the main pancreatic duct, but there was no evidence of any tumors in the pancreas head area. Gastroduodenoscopy revealed a tumor at the papilla of Vater and somatostatinoma was confirmed in the biopsy specimen. Abdominal CT also showed an enhanced tumor 3 cm in diameter, extending from the horizontal part of the duodenum. The patient had a pylorus-preserving pancreaticoduodenectomy and these tumors were diagnosed as somatostatinoma of the papilla of Vater with lymph nodes metastases and duodenal gastrointestinal stromal tumor with positive staining for c-kit and CD34 by immunohistochemistry. There have been several reports about von Recklinghausen's disease patients associated with ampullary somatostatinoma and gastrointestinal stromal tumor, therefore it is necessary to pay attention these tumors as gastrointestinal lesions in these patients.