CC BY-NC-ND 4.0 · Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 2019; 38(04): 342-347
DOI: 10.1055/s-0039-1695761
Case Report | Relato de Caso
Thieme Revinter Publicações Ltda Rio de Janeiro, Brazil

Ependymoma with Intraorbital Extracerebral Recurrence: Case Report

Ependimoma com recidiva extracerebral intraorbital: relato de caso
1   Hospital da Restauração, Recife, PE, Brazil
Benjamim Pessoa Vale
2   Instituto de Neurociências do Piauí, Teresina, PI, Brazil
3   Hospital São Marcos, Teresina, PI, Brazil
Marx Lima de Barros Araújo
2   Instituto de Neurociências do Piauí, Teresina, PI, Brazil
3   Hospital São Marcos, Teresina, PI, Brazil
4   Hospital Universitário, Universidade Federal do Piauí, PI, Brazil
João Cícero Lima Vale
5   Faculdade Facid Wyden, Teresina, PI, Brazil
Yally Dayanne Oliveira Ferreira
6   Centro Universitário Maurício de Nassau, Recife, PE, Brazil
Suelen Maria Silva de Araújo
7   Universidade Católica de Pernambuco, Recife, PE, Brazil
› Author Affiliations
Further Information

Publication History

19 April 2019

01 July 2019

Publication Date:
03 September 2019 (online)


Ependymomas are rare neuroepithelial tumors that originate from a type of glial cell called ependymal cell. In general, they correspond to ∼ 1.2 to 7.8% of all intracranial neoplasms, and to ∼ 2 to 6% of all gliomas. Although it corresponds only to ∼2 to 3% of all primary brain tumors, ependymoma is the fourth most common cerebral neoplasm in children, especially in children younger than 3 years of age.[1] [2] In patients younger than 20 years of age, the majority (90%) of ependymomas are infratentorial, more precisely from the IV ventricle. In spite of this, in adults, medullary ependymomas are more frequent (60%). In this context, supratentorial and extraventricular ependymomas, as in the case reported in the present article, are infrequent in both adults and children.[1] [2] Both sexes are equally affected.[3] Recurrence of intracranial ependymomas occurs in almost 50% of the cases, and the follow-up outcome is not favorable.[4] In another perspective, the recurrence of extracerebral ependymomas is extremely rare, and even more unusual in the intraorbital site, as it occurred in the case in question.


Ependimomas são raros tumores neuroepiteliais originados de um tipo de célula glial chamada célula ependimária. Em geral, correspondem a cerca de 1,2 a7,8% de todas as neoplasias intracraniais, e a cerca de 2 a 6% de todos os gliomas. Embora corresponda apenas a cerca de 2 a 3% de todos os tumores cerebrais primários, o ependimoma é a quarta neoplasia cerebral mais comum em crianças, principalmente nas menores de 3 anos.[1] [2] Em pacientes com menos de 20 anos, a maioria (90%) dos ependimomas são infratentoriais, mais precisamente oriundos do IV ventrículo. A despeito disso, nos adultos são mais frequentes (60%) os ependimomas medulares. Nesse contexto, ependimomas supratentoriais e extraventriculares, como o do caso relatado no presente artigo, são infrequentes tanto em adultos quanto em crianças.[1] [2] Ambos os sexos são afetados igualmente.[3] A recorrência de ependimomas intracraniais ocorre em quase 50% dos casos, sendo o resultado de seu seguimento não muito favorável.[4] Em outra perspectiva, a recorrência extracerebral dos ependimomas é extremamente rara, sendo ainda mais incomum o sítio intraorbital do caso em questão.

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