Brazilian Neurosurgery (Mar 2018)

Supratentorial Atypical Rhabdoid Teratoid Tumor – A Case Report

  • Paulo Moacir Mesquita Filho,
  • Ana Paula Pompeo Vartha,
  • Fernanda De Carli,
  • Gregori Manfroi,
  • Marcelo Pedrotti De Cesaro,
  • Carlos Eduardo Bier Oro,
  • Vitório Ávila Figini,
  • Nério Dutra Azambuja Júnior,
  • Adriana Eli Beck,
  • Daniela Schwingel

DOI
https://doi.org/10.1055/s-0038-1625950
Journal volume & issue
Vol. 37, no. 01
pp. 67 – 70

Abstract

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Atypical rhabdoid teratoid tumors (ARTTs) are rare embryonic tumors, usually localized in the posterior fossa and diagnosed in children under 3 years-old. The treatment includes surgical resection, radio and chemotherapy, and the prognostic is unfavorable, with an average median survival of 1 year. We present the case of a 3-year-old patient, with history of headache and vomiting, followed by absence seizures, temporal automatism, syncope accompanied by sialorrhea and sphincteric loss succeeded by a postictal period. Surgical excision was performed and the anatomopathological study confirmed ARTT. The ARTTs are embryonic tumors, a category in which medulloblastoma and primitive neuroectodermal tumors (PNETs) represent the most common central nervous system (CNS) malignancies in childhood.

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