[A case of multiple gastrointestinal stromal tumors of the small intestine associated with von Recklinghausen disease].

A 63-year-old woman was admitted to our hospital because of tarry stool several years previously. At the time, café-au-lait spots and dermal nodules were found on her entire body, and a diagnosis of von Recklinghausen disease was established. Small bowel endoscopy revealed a submucosal tumor in the jejunum. Laparotomy was therefore performed on the suspicion of intestinal GIST. Numerous extramural tumors with a diameter of 3-5mm were observed along the jejunum and ileum, in addition to the primary tumor. Partial resection of the jejunum, including the primary tumor and only one small nodule was performed to prevent short bowel syndrome. Immunopathological studies of the tumors were positive for KIT and CD34 and we diagnosed multiple intestinal GISTs. Imatinib mesylate was not administered, but no growth of the residual tumors have been recognized for 10 months after surgery.