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Von Recklinghausen's Disease and Gastrointestinal Carcinoids

Lewis Johnson, MD; Michael Weaver, MD
JAMA. 1981;245(24):2496. doi:10.1001/jama.1981.03310490014012.
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To the Editor.—  The occasional association of von Recklinghausen's disease (VRD) with visceral tumors, such as neurofibromas, meningiomas, gliomas, and pheochromocytomas, is well documented1; however, its association with gastrointestinal (GI) carcinoids is limited to only a few case reports.1,2 The purpose of this letter is to document further this association.

Report of a Case.—  A 53-year-old woman with VRD since adolescence was admitted to the hospital for right-sided upperquadrant abdominal pain of 24 hours' duration. Physical examination showed several fleshy pedunculated cutaneous tumors, café-au-lait skin lesions, and a right-sided peroneal palsy. Moderate right-sided upper quadrant abdominal tenderness and guarding were observed. Total serum bilirubin value was 1.9 mg/dL (normal, 0.2 to 1.0 mg/dL) and serum alkaline phosphatase value, 129 units mU/ mL (normal, 30 to 105 mU/mL). Both ultrasonography and an upper GI series examination demonstrated a small tumorlike lesion in the second portion of the duodenum. Exploratory


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