Elsevier

Surgical Neurology

Volume 40, Issue 5, November 1993, Pages 429-434
Surgical Neurology

Primary malignant rhabdoid tumor of the central nervous system: Case report and review of the literature

https://doi.org/10.1016/0090-3019(93)90226-QGet rights and content

Abstract

Malignant rhabdoid tumor (MRT) is well known as a pediatric malignant tumor of the kidney. Only nine cases of primary MRT of the central nervous system (CNS) have been reported. We are reporting in detail the clinical course and treatment of a patient with primary MRT of the CNS.

We cared for a 3-year-old girl with an MRT that extended from the internal auditory canal to the cerebellopontine angle. Despite three surgical attempts at resecting the tumor combined with whole craniospinal axis irradiation, as well as chemotherapy consisting of intravenous nimustine hydrochloride and intrathecal methotrexate injections, the patient died 13 months after her initial hospitalization.

The origin of CNS-MRT development is still a question of pathologic debate. Like renal MRT, the prognosis of MRT of the CNS is very poor. The dissemination of MRT of the CNS occurred in most cases.

References (24)

  • SM Chou et al.

    Primary CNS malignant rhabdoid tumor (MRT): report of two cases and review of literature

    Clin Neuropathol

    (1991)
  • M Ehret et al.

    Embryonal brain neoplasms in the neonatal period and early infancy

    Clin Neuropathol

    (1987)

    • Cited by (0)

    View full text
    Copyright © 1993 Published by Elsevier Inc.