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".