A RARE COMBINATION OF A PHEOCHROMOCYTOMA AND A SOMATOSTATIN-RICH NEUROENDOCRINE TUMOR OF VATERS PAPILLA IN A PATIENT WITH RECKLINGHAUSENS NEUROFIBROMATOSIS

A 74-year-old male suffering from Recklinghausen's fibromatosis (NvR) is reported. He presented with weight loss, cholestasis, endocrine and exocrine pancreatic insufficiency. These symptoms were caused by a ne uroendocrine tumor of the ampulla of Vater containing somatostatin. Th e tumor induced an obstruction of both the common bile and the pancrea tic duct. In addition to this uncommon tumor; a silent pheochromocytom a was found. The patient was treated by endoscopic papillotomy. substi tution of pancreatic enzymes and additional enteral nutrition After re covery no progression of the disease was observed served over one year . a review of the literature shows that patients with neurofibromatosi s are at high risk for periampullar tumors. In particular: somatostati n-rich carcinoids were previously documented. Pheochromocytomas are al so quite prevalent in NvR. However the combination of NvR, pheochromoc ytoma and somatostatin-rich neuroendocrine tumors of the duodenum has only been reported a few times. An explanation for the high prevalence of neuroendocrine tumors in NvR might be the loss of neurofibromin, a tumor suppressor protein, which is the main product of the neurofibro matosis-1-gene.