Intracranial primary nonlymphomatous sarcomas in children: experience with eight cases and review of the literature.

Eight infants and children with intracranial primary nonlymphomatous sarcoma were studied clinically and histologically. Treatment consisted of surgical resection and postoperative radiation therapy. Only one patient received chemotherapy. Two patients are alive after 10 years, but the other six patients died 3 to 33 months after operation. Two illustrative cases with neuroradiological, light microscopic, and ultrastructural findings are presented; both cases showed radiological evidence of intracranial arterial encasement by sarcoma and computed tomographic evidence of multifocal lesions. The pertinent literature is reviewed to illuminate the more refined nature of primary sarcomas of the central nervous system. Irrespective of treatment, the majority of the patients had a limited survival and only a few lived longer than 5 years. Total resection seemed to afford the best prognosis, whereas the efficacy of radiation therapy and chemotherapy is indefinite.