A CASE OF LEIOMYOSARCOMA OF THE JEJUNUM ASSOCIATED WITH VON RECKLINGHAUSEN'S DISEASE

Von Recklinghausen's disease (VRD) is characterized by cafe au lait spots and multiple skin tumors. It may also be accompanied by other systemic lesions. We experienced a case of leiomyosarcoma of the jejunum associated with VRD and report it in this paper with a review of the literature. A 43-year-old man visited our hospital with the chief complaints of epigastraligia and anemia. On physical examination, there were cafe au lait spots over his entire body and skin tumors in the anterior chest and the left upper arm which revealed VRD. The occult blood was strongly positive. There was no abnormality in the stomach, duodenum or colon. Barium meal examination of the small intestine and superior mesenteric angiography showed a myogenic tumor of the jejunum. Partial enterectomy was performed. The histopathological examination showed that it was leiomyosarcoma. There are 23 reported cases of smooth muscle tumors of the gastrointestinal tract with VRD which include the one just reported. In 18 of the 23 cases, the tumors were leiomyoma and in five they were leiomyosarcoma. The possibility of digestive disease should be borne in mind in every patient with VRD and symptoms of digestive dysfunction.