Brain metastasis from clear cell sarcoma of the kidney--a case report and review of the literature.

We describe a 5 year old boy with a 5.5 cm right frontal lobe brain metastasis from primary clear cell sarcoma of the kidney without evidence of tumor-associated edema or contrast enhancement on either computed tomography or magnetic resonance imaging. The metastasis regressed but did not disappear with chemotherapy and dexamethasone, and the residual tumor was removed surgically. On histological examination the majority of the tumor was composed of mature connective tissue with a rim of typical renal clear cell sarcoma cells at the brain-tumor interface. The avascular and desmoplastic nature of the metastasis may explain the unusual radiographic features. Brain metastases from this tumor have only infrequently been reported; therefore a brief review of this rare tumor is provided.