Ependymoma: Evaluation and Management Updates

Purpose of Review To review state of art and relevant advances in the molecular genetics and management of ependymomas of children and adults. Recent Findings Ependymomas may occur either in the brain or in the spinal cord. Compared with intracranial ependymomas, spinal ependymomas are less frequent and exhibit a better prognosis. The new WHO classification of CNS tumors of 2021 has subdivided ependymomas into different histomolecular subgroups with different outcome. The majority of studies have shown a major impact of extent of resection; thus, a complete resection must be performed, whenever possible, at first surgery or at reoperation. Conformal radiotherapy is recommended for grade 3 or incompletely resected grade II tumors. Proton therapy is increasingly employed especially in children to reduce the risk of neurocognitive and endocrine sequelae. Craniospinal irradiation is reserved for metastatic disease. Chemotherapy is not useful as primary treatment and is commonly employed as salvage treatment for patients failing surgery and radiotherapy. Standard treatments are still the mainstay of treatment: the discovery of new druggable pathways will hopefully increase the therapeutic armamentarium in the near future.

Automated summary

This review summarizes the molecular genetics and management advances in ependymomas of children and adults. Spinal ependymomas have a lower occurrence rate and better prognosis compared to intracranial ependymomas. The new WHO classification has categorized ependymomas into different subgroups based on histomolecular characteristics, which have different outcomes. The extent of resection has a major impact on the treatment efficacy, and conformal radiotherapy and chemotherapy are recommended as treatments. Standard treatments are currently the mainstay, but the discovery of new druggable pathways holds promise for future therapeutic options.