Ellison DW, McLendon R, Wiestler OD et al (2016) Ependymal tumors. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Ellison DW, Figarella-Branger D, Perry A, Reifenberger G, von Deimling A (eds) WHO classification of tumours of the central nervous system, 4th edn. International Agency for Research on Cancer, Lyon, pp 101–114

Google Scholar 

McGuire CS, Sainani KL, Fisher PG (2009) Incidence patterns for ependymoma: a surveillance, epidemiology, and end results study. J Neurosurg 110:725–729

Article  Google Scholar 

Ellison DW, Kocak M, Figarella-Branger D et al (2011) Histopathological grading of pediatric ependymoma: reproducibility and clinical relevance in European trial cohorts. J Negat Results Biomed 10:7

Article  Google Scholar 

Tihan T, Zhou T, Holmes E et al (2008) The prognostic value of histological grading of posterior fossa ependymomas in children: a Children’s Oncology Group study and a review of prognostic factors. Mod Pathol 21:165–177

Article  Google Scholar 

Gupta K, Salunke P (2017) Understanding ependymoma oncogenesis: an update on recent molecular advances and current perspectives. Mol Neurobiol 54(1):15–21

CAS  Article  Google Scholar 

Pajtler KW, Witt H, Sill M et al (2015) Molecular classification of ependymal tumors across all CNS compartments, histopathological grades, and age groups. Cancer Cell 27(5):728–743

CAS  Article  Google Scholar 

Ellison DW (2022) Ependymoma. In: WHO classification of tumours editorial board. World Health Organization classification of tumours of the central nervous system. 5th edn. International Agency for Research on Cancer press, Lyon, pp 159–176

Parker M, Mohankumar KM, Punchihewa C et al (2014) C11orf95-RELA fusions drive oncogenic NF-κB signalling in ependymoma. Nature 506(7489):451–455

CAS  Article  Google Scholar 

Pietsch T, Wohlers I, Goschzik T et al (2014) Supratentorial ependymomas of childhood carry C11orf95-RELA fusions leading to pathological activation of the NF-κB signaling pathway. Acta Neuropathol 127(4):609–611

Article  Google Scholar 

Ellison DW, Aldape KD, Capper D et al (2020) cIMPACT-NOW update 7: advancing the molecular classification of ependymal tumors. Brain Pathol 30(5):863–866

PubMed  PubMed Central  Google Scholar 

Andreiuolo F, Varlet P, Tauziède-Espariat A et al (2019) Childhood supratentorial ependymomas with YAP1-MAMLD1 fusion: an entity with characteristic clinical, radiological, cytogenetic and histopathological features. Brain Pathol 29(2):205–216

CAS  Article  Google Scholar 

Torre M, Alexandrescu S, Dubuc AM et al (2020) Characterization of molecular signatures of supratentorial ependymomas. Mod Pathol 33(1):47–56

CAS  Article  Google Scholar 

Malgulwar PB, Nambirajan A, Pathak P et al (2018) C11orf95-RELA fusions and upregulated NF-KB signalling characterise a subset of aggressive supratentorial ependymomas that express L1CAM and nestin. J Neurooncol 138(1):29–39

CAS  Article  Google Scholar 

Gessi M, Giagnacovo M, Modena P et al (2019) Role of immunohistochemistry in the identification of supratentorial C11ORF95-RELA fused ependymoma in routine neuropathology. Am J Surg Pathol 43(1):56–63

Article  Google Scholar 

Sasaki A, Hirato J, Hirose T et al (2019) Review of ependymomas: assessment of consensus in pathological diagnosis and correlations with genetic profiles and outcome. Brain Tumor Pathol 36(2):92–101

Article  Google Scholar 

Pagès M, Pajtler KW, Puget S et al (2019) Diagnostics of pediatric supratentorial RELA ependymomas: integration of information from histopathology, genetics, DNA methylation and imaging. Brain Pathol 29(3):325–335

Article  Google Scholar 

Merchant TE, Bendel AE, Sabin ND et al (2019) Conformal radiation therapy for pediatric ependymoma, chemotherapy for incompletely resected ependymoma, and observation for completely resected, supratentorial ependymoma. J Clin Oncol 37(12):974–983

CAS  Article  Google Scholar 

Fukuoka K, Kanemura Y, Shofuda T et al (2018) Significance of molecular classification of ependymomas: C11orf95-RELA fusion-negative supratentorial ependymomas are a heterogeneous group of tumors. Acta Neuropathol Commun 6(1):134

CAS  Article  Google Scholar 

Witt H, Mack SC, Ryzhova M et al (2011) Delineation of two clinically and molecularly distinct subgroups of posterior fossa ependymoma. Cancer Cell 20(2):143–157

CAS  Article  Google Scholar 

Panwalkar P, Clark J, Ramaswamy V et al (2017) Immunohistochemical analysis of H3K27me3 demonstrates global reduction in group-A childhood posterior fossa ependymoma and is a powerful predictor of outcome. Acta Neuropathol 134(5):705–714

CAS  Article  Google Scholar 

Lim KY, Lee K, Shim Y et al (2022) Molecular subtyping of ependymoma and prognostic impact of Ki-67. Brain Tumor Pathol 39(1):1–13

CAS  Article  Google Scholar 

Ghasemi DR, Sill M, Okonechnikov K et al (2019) MYCN amplification drives an aggressive form of spinal ependymoma. Acta Neuropathol 138(6):1075–1089

CAS  Article  Google Scholar 

Chavali P, Rao S, Palavalasa S et al (2019) L1CAM immunopositivity in anaplastic supratentorial ependymomas: correlation with clinical and histological parameters. Int J Surg Pathol 27(3):251–258

Article  Google Scholar 

Wani K, Armstrong TS, Jones DT et al (2014) Characterization of L1CAM as a clinical marker for the C11orf95-RELA fusion in supratentorial ependymomas. Neuro Oncol 16(suppl 5):v30–v30

Article  Google Scholar 

Figarella-Branger D, Lechapt-Zalcman E, Tabouret E et al (2016) Supratentorial clear cell ependymomas with branching capillaries demonstrate characteristic clinicopathological features and pathological activation of nuclear factor-kappaB signaling. Neuro Oncol 18:919–927

CAS  Article  Google Scholar 

Wang L, Liu L, Li H et al (2019) RELA fusion in supratentorial extraventricular ependymomas: a morphologic, immunohistochemical, and molecular study of 43 cases. Am J Surg Pathol 43(12):1674–1681

Article  Google Scholar 

Zschernack V, Jünger ST, Mynarek M et al (2021) Supratentorial ependymoma in childhood: more than just RELA or YAP. Acta Neuropathol 141(3):455–466

CAS  Article  Google Scholar 

Tauziède-Espariat A, Siegfried A, Nicaise Y et al (2021) Supratentorial non-RELA, ZFTA-fused ependymomas: a comprehensive phenotype genotype correlation highlighting the number of zinc fingers in ZFTA-NCOA1/2 fusions. Acta Neuropathol Commun 9(1):135

Article  Google Scholar 

Upadhyaya SA, Robinson GW, Onar-Thomas A et al (2019) Molecular grouping and outcomes of young children with newly diagnosed ependymoma treated on the multi-institutional SJYC07 trial. Neuro Oncol 21(10):1319–1330

Article  Google Scholar 

Lin GL, Monje M (2020) Understanding the deadly silence of posterior fossa A ependymoma. Mol Cell 78(6):999–1001

CAS  Article  Google Scholar 

Mack SC, Witt H, Piro RM et al (2014) Epigenomic alterations define lethal CIMP-positive ependymomas of infancy. Nature 506(7489):445–450

CAS  Article  Google Scholar 

Jain SU, Do TJ, Lund PJ et al (2019) PFA ependymoma-associated protein EZHIP inhibits PRC2 activity through a H3 K27M-like mechanism. Nat Commun 10(1):2146

Article  Google Scholar 

Michealraj KA, Kumar SA, Kim LJY et al (2020) Metabolic regulation of the epigenome drives lethal infantile ependymoma. Cell 181(6):1329–1345

CAS  Article  Google Scholar 

Gessi M, Capper D, Sahm F et al (2016) Evidence of H3 K27M mutations in posterior fossa ependymomas. Acta Neuropathol 132(4):635–637

Article  Google Scholar 

Pajtler KW, Wen J, Sill M et al (2018) Molecular heterogeneity and CXorf67 alterations in posterior fossa group A (PFA) ependymomas. Acta Neuropathol 136(2):211–226

CAS  Article  Google Scholar