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Histopathologic grading of EPN has always been challenging with poor interobserver reproducibility and lack of correlation between histologic grade and patient outcomes. Recent studies have highlighted that, despite histopathological similarities among variants of EPN at different anatomical sites, they possess site-specific genetic and epigenetic alterations, transcriptional profiles and DNA copy number variations. This has led to a molecular and location-based classification for EPN which has been adopted by the World Health Organization Classification of Central Nervous System Tumors and more accurately risk-stratifies patients than histopathologic grading alone. Given the complexity of this evolving field, the purpose of this paper is to offer a practical approach to the diagnosis of EPN, including the selection of the most appropriate molecular surrogate immunohistochemical stains, basic molecular studies and more sophisticated techniques if needed. The goal is to reach a rapid, sound diagnosis, providing essential information regarding prognosis and guiding clinical decision-making.<\/jats:p>","DOI":"10.1177\/10935266211018928","type":"journal-article","created":{"date-parts":[[2022,2,15]],"date-time":"2022-02-15T23:00:53Z","timestamp":1644966053000},"page":"59-67","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":11,"title":["Ependymal Tumors"],"prefix":"10.1177","volume":"25","author":[{"given":"Mariarita","family":"Santi","sequence":"first","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Children\u2019s Hospital of Philadelphia, Philadelphia, Pennsylvania"},{"name":"Department of Pathology and Laboratory Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Angela N","family":"Viaene","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Children\u2019s Hospital of Philadelphia, Philadelphia, Pennsylvania"},{"name":"Department of Pathology and Laboratory Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cynthia","family":"Hawkins","sequence":"additional","affiliation":[{"name":"Arthur and Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, Toronto, Ontario, Canada"},{"name":"Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada"},{"name":"Department of Pathology, The Hospital for Sick Children, Toronto, Ontario, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2022,2,15]]},"reference":[{"key":"bibr1-10935266211018928","doi-asserted-by":"publisher","DOI":"10.3171\/2008.9.JNS08117"},{"key":"bibr2-10935266211018928","volume-title":"WHO Classification of Tumours of the Central Nervous System","author":"Louis DN","year":"2016"},{"issue":"7","key":"bibr3-10935266211018928","volume":"10","author":"Ellison DW","year":"2011","journal-title":"J Negat Results Biomed"},{"key":"bibr4-10935266211018928","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.3800999"},{"key":"bibr5-10935266211018928","volume-title":"WHO Classification of Tumours of the Central Nervous System","author":"Louis DN","year":"2007"},{"key":"bibr6-10935266211018928","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccell.2015.04.002"},{"key":"bibr7-10935266211018928","doi-asserted-by":"publisher","DOI":"10.1038\/nature13109"},{"key":"bibr8-10935266211018928","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-017-1752-4"},{"key":"bibr9-10935266211018928","doi-asserted-by":"publisher","DOI":"10.1007\/s00381-010-1251-6"},{"key":"bibr10-10935266211018928","volume-title":"WHO Classification of Tumours of the Central Nervous System","author":"Louis","year":"2021"},{"issue":"5","key":"bibr11-10935266211018928","doi-asserted-by":"crossref","first-page":"863","DOI":"10.1111\/bpa.12866","volume":"30","author":"Ellison DW","year":"2020","journal-title":"Brain Pathol"},{"key":"bibr12-10935266211018928","unstructured":"Burger CP, Scheithauer BW.\n                      Tumors of the Central Nervous System; AFIP Atlas of Tumor Pathology Series\n                      . 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