title: Biology and management of ependymomas.
abstract: Ependymomas are rare primary tumors of the central nervous system in children and adults that comprise histologically similar but genetically distinct subgroups. The tumor biology is typically more associated with the site of origin rather than being age-specific. Genetically distinct subgroups have been identified by genomic studies based on locations in classic grade II and III ependymomas. They are supratentorial ependymomas with C11orf95-RELA fusion or YAP1 fusion, infratentorial ependymomas with or without a hypermethylated phenotype (CIMP), and spinal cord ependymomas. Myxopapillary ependymomas and subependymomas have different biology than ependymomas with typical WHO grade II or III histology. Surgery and radiotherapy are the mainstays of treatment, while the role of chemotherapy has not yet been established. An in-depth understanding of tumor biology, developing reliable animal models that accurately reflect tumor molecule features, and high throughput drug screening are essential for developing new therapies. Collaborative efforts between scientists, physicians, and advocacy groups will enhance the translation of laboratory findings into clinical trials. Improvements in disease control underscore the need to incorporate assessment and management of patients' symptoms to ensure that treatment advances translate into improvement in quality of life.
authors.affiliation: Neuro-Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland (J.W., M.R.G.); Department of Family Health, University of Texas Health Science Center at Houston, Houston, Texas (T.S.A.).
authors.forename: Jing
authors.initials: J
authors.lastname: Wu
authors.affiliation: Neuro-Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland (J.W., M.R.G.); Department of Family Health, University of Texas Health Science Center at Houston, Houston, Texas (T.S.A.).
authors.forename: Terri S
authors.initials: TS
authors.lastname: Armstrong
authors.affiliation: Neuro-Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland (J.W., M.R.G.); Department of Family Health, University of Texas Health Science Center at Houston, Houston, Texas (T.S.A.).
authors.forename: Mark R
authors.initials: MR
authors.lastname: Gilbert
mesh_terms: D000818:Animals; D001932:Brain Neoplasms / Q000235:genetics / Q000473:pathology*; D004195:Disease Models, Animal; D004806:Ependymoma / Q000235:genetics / Q000473:pathology*; D018315:Glioma, Subependymal / Q000235:genetics / Q000473:pathology*; D006801:Humans; D011788:Quality of Life; D013120:Spinal Cord Neoplasms / Q000175:diagnosis / Q000473:pathology*
keywords: Collaborative Ependymoma Research Network; ependymoma; management; molecular classification; rare disease