Abstract
Von Recklinghausen's disease (VRD) is characterized by systemic cafe au lait spots of skin and multiple neurofibromas. Besides these charaters it is accompanied by a variety of systemic lesions including diseases of the central nervous system. We report a case of huge retroperitoneal leiomyosarcoma complicated with VRD, with a review of the literature.
A 30-year-old man who had been diagnosed as VRD by a dermatologist two years before was seen at the hospital for further examination after an abdominal tumor was pointed out a medical checkup. Abdominal ultrasonography CT and angiography showed a left retroperitoneal tumor, and a resection of the tumor was performed. The tumor was located laying up the left iliopsoas muscle and was looked like connected to the root of the L2-L4 nerves. We amputated so as not to hurt the nerves. The histopathological and immunohistological examinations showed that it was leiomyosarcoma.
Some cases of leiomyosarcoma of the gastrointestinal tract with VRD have been reported but retroperitoneal leiomyosarcoma is very rarely associated.
