Abstract
Ependymomas are relatively rare glial neoplasms that recapitulate the morphological, immunohistochemical, and ultrastructural features of ependymal cells lining the ventricles and central canal of spinal cord. They present in both children and adults and occur across all three anatomical compartments of the central nervous system: supratentorium, posterior fossa, and spinal cord. According to the World Health Organization classification of brain tumors, they are graded 1–3 with the subtypes myxopapillary ependymoma and subependymoma assigned WHO grade 1, classic ependymoma assigned WHO grade 2, and anaplastic ependymoma assigned WHO grade 3. They are primarily treated by surgery with adjuvant radiotherapy administered in tumors with incomplete resection and/or grade 3 histology. The most common cause of disease failure is local recurrence. WHO histological grade alone is a poor clinical outcome predictor and recent studies have delineated at least nine different site-specific molecular subgroups of ependymomas with distinct demographic, clinicopathological, and (epi)genetic profiles. Particularly among intracranial grade 2/3 ependymomas, molecular subgrouping shows better correlation with outcome than histological grade alone and is emerging as a guide for therapeutic decisions.
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Nambirajan, A., Sharma, A., Sharma, M.C. (2021). Pathology and Molecular Pathology of Ependymoma. In: Mallick, S., Giridhar, P., Rath, G.K. (eds) Evidence based practice in Neuro-oncology. Springer, Singapore. https://doi.org/10.1007/978-981-16-2659-3_4
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