Journal of Neuro-Oncology

, Volume 61, Issue 2, pp 121–126

Primary Malignant Rhabdoid Tumors of the Central Nervous System: Considerations about Two Cases of Adulthood Presentation


  • José Pimentel
    • Laboratory of Neuropathology, Department of NeurologyHospital de Santa Maria
  • Rita Silva
    • Department of NeurologyHospital de Santa Maria
  • Teresa Pimentel
    • Department of NeurologyInstituto Português de Oncologia Francisco Gentil

DOI: 10.1023/A:1022135518846

Cite this article as:
Pimentel, J., Silva, R. & Pimentel, T. J Neurooncol (2003) 61: 121. doi:10.1023/A:1022135518846


Malignant rhabdoid tumors (MRT) are aggressive neoplasms generally of the infant kidney, although a few extrarenal, central nervous system-located, were reported. We describe two cases of such tumors arising intracerebrally in patients aged 16 and 31 years, hence older than the usually reported, with survival times of 16 and 4 months, respectively. Besides, in the youngest, the mass seemed to have developed from a low grade glioma, an occurrence not reported earlier, although the coexistence of morphological features of glioma and rhabdoid tumor is well known. The broad immunoprofile spectrum found in these two tumors seems to reflect the lack of specific differentiation of MRTs. As in the case of other MRTs, our patients had short survivals despite the aggressive post-surgical treatment. In conclusion, MRTs should be suspected also in intracerebral neoplasms of adult patients, and the medical treatment remains disappointing.

brainimmunochemistrymalignant rhabdoid tumortreatment
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© Kluwer Academic Publishers 2003