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Ependymal tumours 

Ependymal tumours
Ependymal tumours

Mark R. Gilbert

and Roberta Rudà

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date: 27 February 2021

Ependymomas are uncommon central nervous system cancers that can arise in the supratentorial, infratentorial, or spinal cord region. Recently, there have been several seminal findings regarding the molecular profiles of ependymomas that have led to marked changes in the classification of this disease. In addition to the World Health Organization grading system that designates ependymomas based on histological appearance into grade I, II, or III, a new molecular classification with distinct entities within the three anatomical regions provides additional subtyping that has prognostic significance and may ultimately provide therapeutic targets. Ependymomas are typically treated with maximum safe tumour resection. Grade III tumours always require radiation treatment even with extensive resection. Radiation is also often administered to patients with grade II ependymomas. Grade I tumours typically receive radiation if there is extensive residual disease, but complete resection may be curative. Local radiation is optimal unless there is imaging or cytological evidence of dissemination in the cerebrospinal fluid. Chemotherapy is less well established although recent molecular findings may lead to subtype specific treatments.

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