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.
Keywords: Chemotherapy; Conformal radiotherapy; MYCN-amplified spinal ependymoma; Myxopapillary ependymoma; Posterior fossa A; Posterior fossa B; Posterior fossa ependymomas; Proton therapy; Spinal cord ependymomas; Stereotactic radiotherapy; Supratentorial ependymomas; Surgery; YAP-1 fusion-positive ependymoma; ZFTA fusion-positive ependymoma.
© 2022. The Author(s).