We experienced a rare case of leiomyosarcoma of the jejunum with von Recklinghausen's disease (VRD). A 59-year-old woman was seen at the hospital because of anemia and epigastraligia. On physical examination, there were cafe au lait spots and multiple skin nodules over the entire body which revealed VRD. Because an abdominal tumor was found around the umbilicus, ultrasonography, X-ray examination of the small intestine, CT and angiography were carried out. They suggested a small dumbbellike tumor (7cm in diameter) of the jejunum. At operation, the intra-extratubal growing tumor was found in the proximal jejunum and a partial enterectomy was performed. The histopathological examination showed that it was leiomyosarcoma.
There are only 30 reported cases of leiomyosarcoma of the gastrointestinal tract with VRD which include the present case just reported. Some notes on the desease in a review of the literature are also presented here.