Supratentorial Atypical Rhabdoid Teratoid Tumor – A Case Report

• Published in: Arquivos brasileiros de neurocirurgia Vol. 37; no. 1; pp. 67 - 70
• Main Authors: Mesquita Filho, Paulo, Vartha, Ana, De Carli, Fernanda, Manfroi, Gregori, De Cesaro, Marcelo, Oro, Carlos, Figini, Vitório, Azambuja Júnior, Nério, Beck, Adriana, Schwingel, Daniela
• Format: Journal Article
• Language: English
• Published: Thieme Revinter Publicações Ltda 01-03-2018
• Subjects: atypical rhabdoid teratoid tumor; embryonic tumors; malignancies in childhood; neuroectodermal tumors
• ISSN: 0103-5355; 2359-5922
• DOI: 10.1055/s-0038-1625950

Abstract
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.