FRANÇOIS PARAF, M.D.; THIERRY POYNARD, M.D., Ph.D.; PIERRE BEDOSSA, M.D.; FRANCOIS GAYRAL, M.D.; SYLVIE NAVEAU, M.D.; JEAN-PIERRE PIGNON, M.D.; GUY LEMAIGRE, M.D.; JEAN-CLAUDE CHAPUT, M.D.
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To the editor: Sprengers and associates (1) report the first two cases of obstructive jaundice related to primary bile duct carcinoma in patients with von Recklinghausen disease. Carcinomas of the gastrointestinal tract, bile duct, and pancreas have been reported in association with neurofibromatosis, but seem under-represented in comparison with the general population (2). We report carcinoma of the Vater's ampulla in a patient with von Recklinghausen disease.
A 65-year-old man with known cutaneous neurofibromatosis was hospitalized because of painless jaundice. His total serum bilirubin level was 366 µmol/L and alkaline phosphatase level, 756 IU/L. Carcinoembryonic antigen level was not increased.
PARAF F, POYNARD T, BEDOSSA P, GAYRAL F, NAVEAU S, PIGNON J, et al. Adenocarcinoma of the Vater's Ampulla and von Recklinghausen Disease. Ann Intern Med. ;107:785. doi: 10.7326/0003-4819-107-5-785_1
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Published: Ann Intern Med. 1987;107(5):785.
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