[Type I neurofibromatosis, pheochromocytoma and somatostatinoma of the ampulla. Literature review].
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.
Caiazzo
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Service de Chirurgie Digestive et Générale, Hôpital C.-Huriez, CHRU, place de Verdun, 59037 Lille cedex, France.
Mariette
C
C
Piessen
G
G
Jany
T
T
Carnaille
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B
Triboulet
J-P
JP
D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D000315:Adrenalectomy; D000328:Adult; D000368:Aged; D014670:Ampulla of Vater; D003138:Common Bile Duct Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis; D010673:Pheochromocytoma / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D013005:Somatostatinoma / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D013997:Time Factors