Rhabdoid tumors of the central nervous system

Rhabdoid tumors of the central nervous system are rare malignancies with a still almost uniformly fatal outcome. There is still no proven curative the rapy available. We report our experience with nine patients with central ne rvous system rhabdoid tumors. Gross complete surgical removal of the tumor was achieved in six patients. Seven patients received intensive chemotherap y. Four of these were treated in addition with both neuroaxis radiotherapy and a local boost directed to the tumor region, while two patients received local radiotherapy only. The therapy was reasonably well tolerated in most cases. Despite the aggressive therapy, eight of the nine patients died fro m progressive tumor disease, and one patient died from hemorrhagic brain st em lesions of unknown etiology. The mean survival time was 10 months after diagnosis. Conventional treatment, although aggressive, cannot change the f atal prognosis of central nervous system rhabdoid tumors. As these neoplasm s are so rare, a coordinated register would probably be a good idea, offeri ng a means of learning more about the tumor's biology and possible strategi es of treatment.