TREATMENT OF PRIMARY MALIGNANT RHABDOID TUMOR OF THE BRAIN - REPORT OF 3 CASES AND REVIEW OF THE LITERATURE

Purpose: Primary malignant rhabdoid tumor (MRT) of the central nervous system is an extremely aggressive tumor predominantly related to earl y childhood, with characteristic histopathological findings but unclea r histogenesis. Owing to its low incidence, little knowledge exists co ncerning the best therapeutic strategy, Methods and Materials: Three c hildren of our hospital with MRT of the brain underwent a maximum tumo r resection followed by multidrug chemotherapy and radiation therapy t o the craniospinal axis, Results: Relapse was disseminated along the s pinal subarachnoid spaces in one child and occurred at the primary tum or site in the other two patients. Maximum survival was 15 months from diagnosis. Conclusion: A review of patients reported in the literatur e and a comparison to our patients reveals a high propensity to early local relapse and meningeal dissemination. In the absence of more effe ctive therapeutic options, we recommend multidisciplinary treatment of patients in good general condition and with resectable disease. In pa rticular, following radiation therapy, tumor remissions and delay of t umor regrowth have been observed. (C) 1998 Elsevier Science Inc.