Malignant rhabdoid tumor of the central nervous system with subarachnoid dissemination

A case of primary central nervous system malignant rhabdoid tumor is presented. Clinical, radiological, and histopathological findings are described in detail. Because of a relatively long clinical course after presentation, it was possible to assess the clinical and radiological response to different treatment modalities: surgery, chemotherapy, and radiotherapy. Despite the complete clinical and radiological response that was achieved after subtotal excision, two cycles of chemotherapy, and high-dose radiotherapy, the tumor recurred within 4 months of completion of the treatment, with wide subarachnoid dissemination. Radiotherapy treatment of whole cranial axis is recommended.