Gastrointestinal involvement in von Recklinghausen's neurofibromatosis

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Abstract
Thirty-nine cases of von Recklinghausen9s neurofibromatosis with gastrointestinal involvement, including seven newly reported cases, are reviewed. Ten percent of patients with von Recklinghausen9s disease at the Massachusetts General Hospital had pathologically proven gastrointestinal neurofibromatosis. Twenty-seven patients had gastrointestinal symptoms prior to discovery of gastrointestinal tumors. The most common sites of involvement were the jejunum (17 cases) and the stomach (16), but multiple tumor sites have been reported. In 30 of the 39 cases the tumors were neurofibromas, and in five they were leiomyomas; in eight the tumor had malignant characteristics. Gastrointestinal tumors should be considered in every patient with von Recklinghausen9s disease and symptoms of gastrointestinal dysfunction.

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