VON RECKLINGHAUSEN'S DISEASE ASSOCIATED WITH LEIOMYOSARKOMA OF THE DUODENUM-A CASE REPORT-

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  • von Recklinghausen病に十二指腸平滑筋肉腫を合併した1例

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Abstract

Association of nonneurogenic neoplasms with von Recklinghausen's disease is not very infrequent, though leiomyosarcoma of the duodenum is rare, and only seven cases have been reported in the Japanese literature. We experienced such a case of von Recklinghausen's disease with duodenal leiomyosarcoma, that is the eighth report.<br> A 49-year-old woman with a history of von Recklinghausen's disease was seen at the hospital because of anemia. Endoscopic examination of the upper digestive tract revealed a mass in the duodenum. An abdominal CT scan and celiac arteriography demonstrated a submucosal tumor in the descending portion of the duodenum. On laparotomy, a mass located opposite the papilla of Vater and growing mainly extramurally was found and was resected locally. Histologic sections of the tumor revealed leiomyosarcoma.<br> It is emphasized that the presence of malignant neoplasm of the digestive tract should be considered in patients with von Recklinghausen's disease who have anemia or gastrointestinal symptoms.

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