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 therapy available. We report our experience with nine patients with central nervous system rhabdoid tumors. Gross complete surgical removal of the tumor...

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Bibliographic Details
Published in:Child's nervous system 2000-04, Vol.16 (4), p.228-234
Main Authors: REINHARDT, D, BEHNKE-MURSCH, J, WEISS, E, CHRISTEN, H.-J, KÜHL, J, LAKOMEK, M, PEKRUN, A
Format: Article
Language:English
Subjects:
Adolescent
Biological and medical sciences
Brain Neoplasms - diagnosis
Brain Neoplasms - mortality
Brain Neoplasms - surgery
Chemotherapy, Adjuvant
Child, Preschool
Combined Modality Therapy
Female
Follow-Up Studies
Humans
Infant
Male
Medical sciences
Neoplasm Recurrence, Local - diagnosis
Neoplasm Recurrence, Local - mortality
Neurosurgery
Radiotherapy, Adjuvant
Rhabdoid Tumor - diagnosis
Rhabdoid Tumor - mortality
Rhabdoid Tumor - surgery
Skull, brain, vascular surgery
Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases
Survival Rate
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Summary:Rhabdoid tumors of the central nervous system are rare malignancies with a still almost uniformly fatal outcome. There is still no proven curative therapy available. We report our experience with nine patients with central nervous system rhabdoid tumors. Gross complete surgical removal of the tumor was achieved in six patients. Seven patients received intensive chemotherapy. 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 from progressive tumor disease, and one patient died from hemorrhagic brain stem lesions of unknown etiology. The mean survival time was 10 months after diagnosis. Conventional treatment, although aggressive, cannot change the fatal prognosis of central nervous system rhabdoid tumors. As these neoplasms are so rare, a coordinated register would probably be a good idea, offering a means of learning more about the tumor's biology and possible strategies of treatment.
ISSN:0256-7040
1433-0350
DOI:10.1007/s003810050503