Ependymoma

Abstract

Ependymoma can arise throughout the whole neuraxis. In children, tumors predominantly occur intracranially, whereas the spine is the most prevalent location in adults. Significant variance in the grade II versus grade III distinction of ependymomas has led to the acknowledgment that the clinical utility of histopathological classification is limited. Epigenomic profiling efforts have identified molecularly distinct groups of ependymomas that adequately reflect the biological, clinical, and histopathological heterogeneities across anatomical compartments, age groups, and grades. The recent update of the World Health Organization classification of central nervous system tumors has already integrated one of these groups, and molecular classification will be part of future clinical trials to improve risk stratification. Clinical management of this rare disease is challenging, making professional experience and intensified multidisciplinary cooperation pivotal factors for treatment success. Novel research strategies are currently applied for target discovery in ependymomas since for most molecular groups, genetic drivers are unknown.

Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review

“…The role of chemotherapy in treating children affected by intracranial ependymomas remains unproven despite intensive investigation [52,53]. Thus, it is not surprising that its use in the management of the extra CNS counterpart is still a matter of debate.…”

Perfil Anatomopatológico e Imuno-histoquímico de Gliomas de Pacientes da Região de Maringá-PR

“…O RELA-fusion é o mais importante teste molecular dos ependimomas preconizado atualmente, embora com implicação clínica limitada. O L1 cell adhesion molecule (L1CAM) ainda carece de estudos, sua expressão em ependimomas supratentoriais está associada ao RELA--fusion, o que indica pior prognóstico 1,24 . Apenas dois tumores com RELA-fusion foram testados, ambos com positividade.…”

“…Também foi observada, neste estudo, uma maior percentagem de pacientes do sexo masculino (64,3%). Apesar de ocorrer em qualquer idade, a maioria dos ependimomas se apresenta na população pediátrica 24 , o que não ocorreu neste estudo, possivelmente em razão da baixa amostragem desse subtipo.…”

An unexpected diagnosis of paediatric subcutaneous sacrococcygeal extraspinal ependymoma: lessons learnt and review of the literature

“…They arise from the ependymal cells lining the ventricular system of the central nervous system, which are responsible for regulating the composition of cerebrospinal fluid [ 2 ]. Ependymomas can therefore be found anywhere along the neuraxis and appear to have a bimodal age presentation, most commonly occurring in the first and fourth decades [ 3 ].…”

“…Although uncommon in adults (accounting for only ~2% of all adult primary central nervous system tumours), ependymomas in this population arise most commonly in the spine. However in the paediatric population 90% of ependymomas are seen intracranially, especially in the posterior fossa [ 3 ]. Rarely, ependymomas have been reported to arise extraspinally, most commonly as sacrococcygeal tumours, and the pathogenesis of how the tumour arises outside of the neuraxis, in the apparent absence of ependymal cells, is poorly understood [ 1 ].…”