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[Type I neurofibromatosis, pheochromocytoma and somatostatinoma of the ampulla. Literature review].
Annales de Chirurgie 2006 July
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.
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