Abstract

Some patients with von Recklinghausen's disease are accompanied with skin neoplasms and varieties of other systemic lesions. We experienced a rare case of leiomoysarcoma of the duodenum complicated with von Recklinghausen's disease, and we reported it with the review of other reports.A-62-year-old female was admitted to the hospital because of general fatigue. Many cafe-lait spots and skin nodules were found, and abdominal tumor with severe anemia was also detected. After UGI, CT, and angiography were done the operation was performed under the diagnosis of leiomyosarcoma of the duodenum. The tumor was originated from the third portion of the duodenum and grew extramurally, so partial resection of the duodenum and the jejunum performed. This tumor was histologically diagnosed as leiomyosarcoma.Twelve cases of gastrointestinal leiomyosarcoma with von Recklinghausen's disease were reported in Japan. And this case was the third one having leiomyosarcoma originated from the duodenum. The possibility of having digestive disease must be taken into consideration when a patient of von Recklinghausen's disease complains of digestive dysfunction.

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