title: Advances in Management of Pediatric Ependymomas.
abstract: Ependymomas are a heterogeneous group of neuroepithelial tumors of children and adults. In pediatric cases, the standard of care has long consisted of neurosurgical resection to the greatest extent acceptable followed by adjuvant involved field irradiation. Complete macroscopic surgical resection has remained the only consistent clinical variable known to improve survival. Adjuvant chemotherapy has yet to predictably affect outcome, possibly due to the molecular heterogeneity of histologically similar tumors. The administration of chemotherapy subsequently remains limited to clinical trials. However, recent comprehensive genomic, transcriptomic, and epigenetic interrogations of ependymomas have uncovered unique molecular characteristics and subtypes that correlated with clinical features such as age, neuroanatomical location, and prognosis. These findings represent a potential paradigm shift and provide a biologic rationale for targeted therapeutic strategies and risk-adapted administration of conventional treatment modalities. In this review, we focus on intracranial WHO grade II and III ependymoma of children and discuss conventional management strategies, followed by recent biologic findings and novel therapeutics currently under investigation.
authors.affiliation: Section of Hematology-Oncology, Department of Pediatrics, Texas Children's Cancer Center, Baylor College of Medicine, Houston, TX, USA. fylin@txch.org.|Brain Tumor Program, Texas Children's Cancer Center, 6701 Fannin St, Ste 1510, Houston, TX, 77030, USA. fylin@txch.org.|Dan L. Duncan Cancer Center, Houston, TX, USA. fylin@txch.org.
authors.forename: Frank Y
authors.initials: FY
authors.lastname: Lin
authors.affiliation: Section of Hematology-Oncology, Department of Pediatrics, Texas Children's Cancer Center, Baylor College of Medicine, Houston, TX, USA.|Brain Tumor Program, Texas Children's Cancer Center, 6701 Fannin St, Ste 1510, Houston, TX, 77030, USA.|Dan L. Duncan Cancer Center, Houston, TX, USA.
authors.forename: Murali
authors.initials: M
authors.lastname: Chintagumpala
mesh_terms: D001932:Brain Neoplasms / Q000235:genetics / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D017024:Chemotherapy, Adjuvant / Q000639:trends; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D004806:Ependymoma / Q000235:genetics / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D005819:Genetic Markers; D006801:Humans; D007223:Infant; D019635:Neurosurgical Procedures / Q000639:trends*; D011379:Prognosis; D018714:Radiotherapy, Adjuvant / Q000639:trends
chemical_list: D005819:Genetic Markers
keywords: Molecular subtypes of ependymoma; Pediatric ependymoma; Prognostic factors in ependymoma; Radiation therapy for ependymoma