[A case of angiosarcoma with von Recklinghausen's disease]

We treated a patient with von Recklinghausen's disease and angiosarcoma. This combination has not been reported in Japan to the best of our knowledge. An emergency operation for massive gastrointestinal bleeding was performed on a 68-year-old woman with von Recklinghausen's disease, who had been known to have a retroperitoneal tumor; The bleeding was controlled and the tumor was also resected. Postoperative course was uneventful also but one month after operation intra-abdominal and intrathoracic bleeding developed, and the patient died of respiratory failure. Autopsy showed hemorrhagic lesions, which were diagnosed pathologically metastatic angiosarcoma. The possibility of hemorrhagic lesions in the patients with von Recklinghausen's disease should be kept in mind.