Malignant Rhabdoid Tumor of Brain: An Aggressive Clinical Entity

     BJ Fisher, J Siddiqui, D Macdonald, AE Cairney, D Ramsey, D Munoz and R Del Maestro

Abstract:   Objective: We report three patients with malignant rhabdoid tumor (MRT) of the brain, two children and an adult. There were three purposes to this report: to describe the clinical course in an adult with MRT; to describe the interesting histopathological metamorphosis of one of the tumors; and to report the outcome of the treatment regimens we used in order to help guide future treatment. Since these tumors are quite rare it is important to continue to try new regimens in the search for effective therapy rather than to repeat ineffective ones. Method: Report of three patients. Results: The clinical course in all three patients was typical of these aggressive neoplasms in that chemotherapy and radiotherapy were ineffective in modifying the rapid deterioration leading to death. Conclusions: MRT can occur in adults. Autopsy in one patient showed that the tumor seemed to undergo an evolution in appearance when compared with the original pathology specimen from craniotomy. Administration of systemic therapy should be prompt and include intrathecal chemotherapy.

Can. J. Neurol. Sci. 1996; 23: 257-263