[Type I neurofibromatosis, pheochromocytoma and somatostatinoma of the ampulla. Literature review]

Abstract

Gastrointestinal involvement in Von Recklinghausen's neurofibromatosis (autosomal dominant disease) is observed in 25% of patients and is frequently represented by duodenal carcinoids. We report a case of somatostatinoma of the ampulla in a 42-year-old female with neurofibromatosis and pheochromocytoma and show a literature review about the association of neurofibromatosis and somatostatinoma. This pathological association appears non hasardous and surgical ampullectomy offers a good alternative to pancreaticoduodenectomy in localised tumors treatment.