Ependymomas

Overview

Journal Pathologica

Specialty Pathology

Date 2022 Dec 19

PMID 36534422

Authors

Luca Bertero

Alessia Andrea Ricci

Cristian Tampieri

Paola Cassoni

Piergiorgio Modena

Affiliations

Soon will be listed here.

Abstract

Ependymal neoplasms are a heterogenous group of neoplasms arising from the progenitors of the cells lining the ventricular system and the spinal central canal. During the last few years, significant novel data concerning oncogenesis, molecular characteristics and clinical correlations of these tumours have been collected, with a strong relevance for their pathological classification. The recently published 5th edition of WHO Classification of Central Nervous System Tumours integrates this novel knowledge and represents a substantial update compared to the previous edition. Concerning supratentorial ependymomas, the previous fusion-positive ependymoma has been renamed into fusion-positive and the novel fusion-positive ependymoma subtype has been added. Posterior fossa ependymomas should now be allocated either to the Type A or Type B subtypes based on molecular profiling or using the H3 K27me3 immunohistochemical surrogate. Regarding spinal ependymomas, a novel subtype has been added based on a distinctive molecular trait, presence of amplification, and on the unfavourable outcome. Finally, myxopapillary ependymoma is now classified as a grade 2 tumour in accordance with its overall prognosis which mirrors that of conventional spinal ependymomas. The aim of this review is to present these changes and summarize the current diagnostic framework of ependymal tumours, according to the most recent updates.

Citing Articles

Intradural extramedullary double primary ependymoma and meningioma rare condition: Case report and literature review.

Yuan X, Li R, Liu Q Medicine (Baltimore). 2025; 104(2):e41210.

PMID: 39792750 PMC: 11729158. DOI: 10.1097/MD.0000000000041210.

Intracranial ependymoma: A retrospective analysis of clinical features, treatment modalities, and long-term outcome.

Badary A, Kurdi S, Almealawy Y, Alrubaye S, Sanker V, Chaurasia B Aging Med (Milton). 2025; 7(6):679-688.

PMID: 39777089 PMC: 11702391. DOI: 10.1002/agm2.12378.

Purines and purinergic receptors in primary tumors of the central nervous system.

Soares A, Dos Santos H, Domann K, Alves N, Bohm B, Haack C Purinergic Signal. 2024; .

PMID: 39352574 DOI: 10.1007/s11302-024-10053-8.

Supratentorial and Infratentorial Ependymoma.

Myseros J Adv Tech Stand Neurosurg. 2024; 53:93-118.

PMID: 39287805 DOI: 10.1007/978-3-031-67077-0_7.

Ventricular system-unrelated cerebellar ependymoma: A case report.

Yang C, Xue R, Yang L, Jieda X, Xiang W, Zhou J World J Clin Cases. 2024; 12(25):5814-5820.

PMID: 39247726 PMC: 11263058. DOI: 10.12998/wjcc.v12.i25.5814.

References

Bates J, Choi G, Milano M . Myxopapillary ependymoma: a SEER analysis of epidemiology and outcomes. J Neurooncol. 2016; 129(2):251-8. DOI: 10.1007/s11060-016-2167-0. View

Massimino M, Barretta F, Modena P, Witt H, Minasi S, Pfister S . Second series by the Italian Association of Pediatric Hematology and Oncology of children and adolescents with intracranial ependymoma: an integrated molecular and clinical characterization with a long-term follow-up. Neuro Oncol. 2020; 23(5):848-857. PMC: 8099475. DOI: 10.1093/neuonc/noaa257. View

Boonyawat B, Charoenpitakchai M, Suwanpakdee P . A First Case Report of Subependymoma in Mutation-Associated Noonan Syndrome. Case Rep Neurol Med. 2019; 2019:6091059. PMC: 6766114. DOI: 10.1155/2019/6091059. View

Pajtler K, Wen J, Sill M, Lin T, Orisme W, Tang B . Molecular heterogeneity and CXorf67 alterations in posterior fossa group A (PFA) ependymomas. Acta Neuropathol. 2018; 136(2):211-226. PMC: 6105278. DOI: 10.1007/s00401-018-1877-0. View

Pajtler K, Witt H, Sill M, Jones D, Hovestadt V, Kratochwil F . Molecular Classification of Ependymal Tumors across All CNS Compartments, Histopathological Grades, and Age Groups. Cancer Cell. 2015; 27(5):728-43. PMC: 4712639. DOI: 10.1016/j.ccell.2015.04.002. View

Varma A, Giraldi D, Mills S, Brodbelt A, Jenkinson M . Surgical management and long-term outcome of intracranial subependymoma. Acta Neurochir (Wien). 2018; 160(9):1793-1799. PMC: 6105212. DOI: 10.1007/s00701-018-3570-4. View

Ragazzini R, Perez-Palacios R, Baymaz I, Diop S, Ancelin K, Zielinski D . EZHIP constrains Polycomb Repressive Complex 2 activity in germ cells. Nat Commun. 2019; 10(1):3858. PMC: 6710278. DOI: 10.1038/s41467-019-11800-x. View

Pages M, Pajtler K, Puget S, Castel D, Boddaert N, Tauziede-Espariat A . Diagnostics of pediatric supratentorial RELA ependymomas: integration of information from histopathology, genetics, DNA methylation and imaging. Brain Pathol. 2018; 29(3):325-335. PMC: 7379587. DOI: 10.1111/bpa.12664. View

Katz S, Cachia D, Kamiya-Matsuoka C, Olar A, Theeler B, Penas Prado M . Ependymomas arising outside of the central nervous system: A case series and literature review. J Clin Neurosci. 2017; 47:202-207. DOI: 10.1016/j.jocn.2017.10.026. View

10.

Pajtler K, Wei Y, Okonechnikov K, Silva P, Vouri M, Zhang L . YAP1 subgroup supratentorial ependymoma requires TEAD and nuclear factor I-mediated transcriptional programmes for tumorigenesis. Nat Commun. 2019; 10(1):3914. PMC: 6718408. DOI: 10.1038/s41467-019-11884-5. View

11.

Sayegh E, Aranda D, Kim J, Oh T, Parsa A, Oh M . Prognosis by tumor location in adults with intracranial ependymomas. J Clin Neurosci. 2014; 21(12):2096-101. PMC: 4474735. DOI: 10.1016/j.jocn.2014.05.011. View

12.

Nishimura H, Fukami S, Endo K, Suzuki H, Sawaji Y, Seki T . A Case of Rapidly-Progressing Cervical Spine Subependymoma with Atypical Features. Spine Surg Relat Res. 2019; 3(1):91-94. PMC: 6690116. DOI: 10.22603/ssrr.2018-0005. View

13.

Pietsch T, Wohlers I, Goschzik T, Dreschmann V, Denkhaus D, Dorner E . Supratentorial ependymomas of childhood carry C11orf95-RELA fusions leading to pathological activation of the NF-κB signaling pathway. Acta Neuropathol. 2014; 127(4):609-11. DOI: 10.1007/s00401-014-1264-4. View

14.

Cavalli F, Hubner J, Sharma T, Luu B, Sill M, Zapotocky M . Heterogeneity within the PF-EPN-B ependymoma subgroup. Acta Neuropathol. 2018; 136(2):227-237. PMC: 6373486. DOI: 10.1007/s00401-018-1888-x. View

15.

Louis D, Wesseling P, Paulus W, Giannini C, Batchelor T, Cairncross J . cIMPACT-NOW update 1: Not Otherwise Specified (NOS) and Not Elsewhere Classified (NEC). Acta Neuropathol. 2018; 135(3):481-484. DOI: 10.1007/s00401-018-1808-0. View

16.

Mack S, Witt H, Piro R, Gu L, Zuyderduyn S, Stutz A . Epigenomic alterations define lethal CIMP-positive ependymomas of infancy. Nature. 2014; 506(7489):445-50. PMC: 4174313. DOI: 10.1038/nature13108. View

17.

Fischer S, Attenhofer M, Gultekin S, Ross D, Heinimann K . TRPS1 gene alterations in human subependymoma. J Neurooncol. 2017; 134(1):133-138. DOI: 10.1007/s11060-017-2496-7. View

18.

Junger S, Andreiuolo F, Mynarek M, Wohlers I, Rahmann S, Klein-Hitpass L . CDKN2A deletion in supratentorial ependymoma with RELA alteration indicates a dismal prognosis: a retrospective analysis of the HIT ependymoma trial cohort. Acta Neuropathol. 2020; 140(3):405-407. PMC: 7423858. DOI: 10.1007/s00401-020-02169-z. View

19.

Rushing E, Cooper P, Quezado M, Begnami M, Crespo A, Smirniotopoulos J . Subependymoma revisited: clinicopathological evaluation of 83 cases. J Neurooncol. 2007; 85(3):297-305. DOI: 10.1007/s11060-007-9411-6. View

20.

Lee J, Sharifai N, Dahiya S, Kleinschmidt-DeMasters B, Rosenblum M, Reis G . Clinicopathologic features of anaplastic myxopapillary ependymomas. Brain Pathol. 2018; 29(1):75-84. PMC: 7444646. DOI: 10.1111/bpa.12673. View