[A case of alveolar soft-part sarcoma with cerebral metastases]

Abstract

A case of alveolar soft-part sarcoma with multiple cerebral metastases in addition to multiple lung and bone metastases is reported. This is a rare tumor which thus far has only been recognized as a malignant soft tissue tumor with great uncertainty. A 24-year-old man with a chief complaint of left frontal headache was admitted to our unit on September 26, 1994. A tumor of the right forearm had been surgically removed 9 years previously, and surgery had been followed by two courses of chemotherapy with vincristine, cyclophosphamide and actinomycin-D and subsequent irradiation for bone metastasis. The chemotherapy, however, did not have any clear effect. Neurological examination at the time revealed only bilateral papilledema, but CT and MRI scan of the brain showed a round mass in the left occipital lobe with homogeneous enhancement and another small mass was detected in the right occipital lobe. A cerebral angiogram showed a vascular tumor. The preoperative diagnosis was multiple cerebral metastases of alveolar soft part sarcoma. Left occipital craniotomy was performed on October 12, 1994, and the tumor was completely removed by lobectomy. Specimens of the tumor showed typical histological features of alveolar soft-part sarcoma. Whole-brain radiotherapy was administered postoperatively, and the patient is well neurologically without right homonymous hemianopsia. The clinical features of alveolar soft-part sarcoma are discussed. Only a few cases have been reported in the literature, but the most frequent site of the primary lesion has been the lower extremities, and cerebral metastasis has not been rare. All efforts should be focused on discovering this tumor in the early stage because surgical removal can yield good results, and radiotherapy is thought to be effective in terms of patient "quality of life".