{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T06:47:59Z","timestamp":1792219679353,"version":"4.4.0"},"reference-count":52,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2022,3,21]],"date-time":"2022-03-21T00:00:00Z","timestamp":1647820800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Brain Pathology"],"published-print":{"date-parts":[[2022,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Ependymal neoplasms occur at all ages and encompass multiple tumor types and subtypes that develop in the supratentorial compartment, the posterior fossa, or the spinal cord. Clinically, ependymomas represent a very heterogeneous group of tumors from rather benign subependymomas to very aggressive and often deadly childhood ependymomas of the posterior fossa. Newly identified biological markers and classification schemes, e.\u00a0g. based on global DNA methylation profiling, have led to the definition of 10\u00a0types of ependymal tumors and an improved prediction of patients\u2019 outcome by applying the new classification system. While the exact genetic basis for several ependymoma types still remains unclear, the knowledge about ependymoma driving events has significantly increased within the last decade and contributed to a classification based on molecular characteristics and localization rather than histological features alone. Convincing evidence is now pointing towards gene fusions involving\n                    <jats:italic>ZFTA<\/jats:italic>\n                    or\n                    <jats:italic>YAP1<\/jats:italic>\n                    causing the development of supratentorial ependymomas. Also, H3,\n                    <jats:italic>EZHIP<\/jats:italic>\n                    , or\n                    <jats:italic>TERT<\/jats:italic>\n                    mutations have been detected in a fraction of infratentorial ependymal tumors. Finally,\n                    <jats:italic>MYCN<\/jats:italic>\n                    amplifications have recently been identified in spinal ependymomas, in addition to the previously known mutations in\n                    <jats:italic>NF2<\/jats:italic>\n                    . This review summarizes how recent findings regarding biology, molecular tumor typing, and clinical outcome have impacted the classification of ependymomas as suggested by the updated 2021\u00a0WHO CNS tumor classification system. We focus on changes compared to the previous classification of 2016 and discuss how a formal grading could evolve in the future and guide clinicians to treat ependymoma patients.\n                  <\/jats:p>","DOI":"10.1111\/bpa.13068","type":"journal-article","created":{"date-parts":[[2022,3,21]],"date-time":"2022-03-21T04:17:03Z","timestamp":1647836223000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":118,"title":["Updates in the classification of ependymal neoplasms: The 2021 WHO Classification and beyond"],"prefix":"10.1111","volume":"32","author":[{"given":"Catena","family":"Kresbach","sequence":"first","affiliation":[{"name":"Department of Pediatric Hematology and Oncology University Medical Center Hamburg\u2010Eppendorf  Hamburg Germany"},{"name":"Research Institute Children\u2019s Cancer Center Hamburg  Hamburg Germany"},{"name":"Institute of Neuropathology University Medical Center Hamburg\u2010Eppendorf  Hamburg 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