Abstract

CAEIRO, Cláudia et al. Atypical Teratoid Rhabdoid Tumor in an Adult. Arq Med [online]. 2008, vol.22, n.6, pp.165-167. ISSN 2183-2447.

Atypical teratoid rhabdoid tumor (AT/RT) is a rare and aggressive malignancy, which is more common in early childhood and carries a poor prognosis. Only 23 cases have been described in adults. A treatment strategy is yet to be established but gross total resection (GTR), early radiotherapy (RT) and platinum and alkylator-based chemotherapy seem to be associated with better outcome. We report on a 39 year-old female who presented with headache, vertigo, blurry vision and ataxia. The MRI revealed hydrocephalus related to a mass at the pineal region. The treatment consisted of GTR, RT and 3 cycles of an outpatient-based ICE regimen. The patient remains disease-free 30 months after diagnosis. Following this case, a review of the literature is undertaken.

Keywords : atypical teratoid rhabdoid tumor; INI1 gene; monosomy 22; chemotherapy.

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