Ependymoma from Benign to Highly Aggressive Diseases: A Review.
Chemotherapy
Ependymoma
Genetics
Neuropathology
Pediatric
Radiation
Journal
Advances and technical standards in neurosurgery
ISSN: 0095-4829
Titre abrégé: Adv Tech Stand Neurosurg
Pays: United States
ID NLM: 7501064
Informations de publication
Date de publication:
2024
2024
Historique:
medline:
9
4
2024
pubmed:
9
4
2024
entrez:
9
4
2024
Statut:
ppublish
Résumé
Ependymomas comprise biologically distinct tumor types with respect to age distribution, (epi)genetics, localization, and prognosis. Multimodal risk-stratification, including histopathological and molecular features, is essential in these biologically defined tumor types. Gross total resection (GTR), achieved with intraoperative monitoring and neuronavigation, and if necessary, second-look surgery, is the most effective treatment. Adjuvant radiation therapy is mandatory in high-risk tumors and in case of residual tumor. There is yet growing evidence that some ependymal tumors may be cured by surgery alone. To date, the role of chemotherapy is unclear and subject of current studies.Even though standard therapy can achieve reasonable survival rates for the majority of ependymoma patients, long-term follow-up still reveals a high probability of relapse in certain biological entities.With increasing knowledge of biologically distinct tumor types, risk-adapted adjuvant therapy gains importance. Beyond initial tumor control, and avoidance of therapy-induced morbidity for low-risk patients, intensified treatment for high-risk patients comprises another challenge. With identification of specific risk features regarding molecular alterations, targeted therapy may represent an option for individualized treatment modalities in the future.
Identifiants
pubmed: 38592527
doi: 10.1007/978-3-031-53578-9_2
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
31-62Informations de copyright
© 2024. The Author(s), under exclusive license to Springer Nature Switzerland AG.
Références
Elsamadicy AA, Koo AB, David WB, et al. Comparison of epidemiology, treatments, and outcomes in pediatric versus adult ependymoma. Neurooncol Adv. 2020;2:vdaa019.
pubmed: 32642681
pmcid: 7212900
McGuire CS, Sainani KL, Fisher PG. Incidence patterns for ependymoma: a surveillance, epidemiology, and end results study. J Neurosurg. 2009;110:725–9.
pubmed: 19061350
doi: 10.3171/2008.9.JNS08117
Ostrom QT, Gittleman H, Liao P, Vecchione-Koval T, Wolinsky Y, Kruchko C, Barnholtz-Sloan JS. CBTRUS statistical report: primary brain and other central nervous system tumors diagnosed in the United States in 2010–2014. Neuro Oncol. 2017;19:v1–v88.
pubmed: 29117289
pmcid: 5693142
doi: 10.1093/neuonc/nox158
Massimino M, Barretta F, Modena P, et al. The AIEOP 2nd series of children and adolescents intracranial ependymoma. An integrated molecular and clinical characterization with a long-term follow-up. Neuro Oncol. 2020;23:848. https://doi.org/10.1093/neuonc/noaa257 .
doi: 10.1093/neuonc/noaa257
pmcid: 8099475
Massimino M, Miceli R, Giangaspero F, et al. Final results of the second prospective AIEOP protocol for pediatric intracranial ependymoma. Neuro Oncol. 2016;18:1451–60.
pubmed: 27194148
pmcid: 5035526
doi: 10.1093/neuonc/now108
Merchant TE, Bendel AE, Sabin ND, et al. Conformal radiation therapy for pediatric ependymoma, chemotherapy for incompletely resected ependymoma, and observation for completely resected, supratentorial ependymoma. J Clin Oncol. 2019;37:974–83.
pubmed: 30811284
pmcid: 7186586
doi: 10.1200/JCO.18.01765
Merchant TE, Li C, Xiong X, Kun LE, Boop FA, Sanford RA. Conformal radiotherapy after surgery for paediatric ependymoma: a prospective study. Lancet Oncol. 2009;10:258–66.
pubmed: 19274783
pmcid: 3615425
doi: 10.1016/S1470-2045(08)70342-5
Ellison DW, Kocak M, Figarella-Branger D, et al. Histopathological grading of pediatric ependymoma: reproducibility and clinical relevance in European trial cohorts. J Negat Results Biomed. 2011;10 https://doi.org/10.1186/1477-5751-10-7 .
Tihan T, Zhou T, Holmes E, Burger PC, Ozuysal S, Rushing EJ. 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. 2008;21:165–77.
pubmed: 18084249
doi: 10.1038/modpathol.3800999
Andreiuolo F, Varlet P, Tauziède-Espariat A, et al. Childhood supratentorial ependymomas with YAP1-MAMLD1 fusion: an entity with characteristic clinical, radiological, cytogenetic and histopathological features. Brain Pathol (Zurich, Switz). 2019;29:205–16.
doi: 10.1111/bpa.12659
Dyer S, Prebble E, Davison V, Davies P, Ramani P, Ellison D, Grundy R. Genomic imbalances in pediatric intracranial ependymomas define clinically relevant groups. Am J Pathol. 2002;161:2133–41.
pubmed: 12466129
pmcid: 1850918
doi: 10.1016/S0002-9440(10)64491-4
Kilday J-P, Mitra B, Domerg C, et al. Copy number gain of 1q25 predicts poor progression-free survival for pediatric intracranial ependymomas and enables patient risk stratification: a prospective European clinical trial cohort analysis on behalf of the Children’s Cancer Leukaemia Group (CCLG), Societe Francaise d’Oncologie Pediatrique (SFOP), and International Society for Pediatric Oncology (SIOP). Clin Cancer Res. 2012;18:2001–11.
pubmed: 22338015
doi: 10.1158/1078-0432.CCR-11-2489
Korshunov A, Witt H, Hielscher T, et al. Molecular staging of intracranial ependymoma in children and adults. J Clin Oncol. 2010;28:3182–90.
pubmed: 20516456
doi: 10.1200/JCO.2009.27.3359
Mack SC, Witt H, Piro RM, et al. Epigenomic alterations define lethal CIMP-positive ependymomas of infancy. Nature. 2014;506:445–50.
pubmed: 24553142
pmcid: 4174313
doi: 10.1038/nature13108
Pajtler KW, Witt H, Sill M, et al. Molecular classification of ependymal tumors across all CNS compartments, histopathological grades, and age groups. Cancer Cell. 2015;27:728–43.
pubmed: 25965575
pmcid: 4712639
doi: 10.1016/j.ccell.2015.04.002
Parker M, Mohankumar KM, Punchihewa C, et al. C11orf95–RELA fusions drive oncogenic NF-κB signalling in ependymoma. Nature. 2014;506:451–5.
pubmed: 24553141
pmcid: 4050669
doi: 10.1038/nature13109
Taylor MD, Poppleton H, Fuller C, et al. Radial glia cells are candidate stem cells of ependymoma. Cancer Cell. 2005;8:323–35.
pubmed: 16226707
doi: 10.1016/j.ccr.2005.09.001
WHO Classification of Tumours Editorial Board. World Health Organization classification of tumours of the central nervous system. 5th ed. Lyon: International Agency for Research on Cancer; 2021.
Witt H, Mack SC, Ryzhova M, et al. Delineation of two clinically and molecularly distinct subgroups of posterior fossa ependymoma. Cancer Cell. 2011;20:143–57.
pubmed: 21840481
pmcid: 4154494
doi: 10.1016/j.ccr.2011.07.007
Jünger ST, Andreiuolo F, Mynarek M, Dörner E, Zur Mühlen A, Rutkowski S, von Bueren AO, Pietsch T. Ependymomas in infancy: underlying genetic alterations, histological features, and clinical outcome. Childs Nerv Syst. 2020;36:2693. https://doi.org/10.1007/s00381-020-04655-x .
doi: 10.1007/s00381-020-04655-x
pubmed: 32474813
pmcid: 7575464
Kilday J-P, Rahman R, Dyer S, Ridley L, Lowe J, Coyle B, Grundy R. Pediatric ependymoma: biological perspectives. Mol Cancer Res. 2009;7:765–86.
pubmed: 19531565
doi: 10.1158/1541-7786.MCR-08-0584
Witt H, Gramatzki D, Hentschel B, et al. DNA methylation-based classification of ependymomas in adulthood: implications for diagnosis and treatment. Neuro Oncol. 2018;20:1616–24.
pubmed: 30053291
pmcid: 6231197
doi: 10.1093/neuonc/noy118
Nowak J, Jünger ST, Huflage H, Seidel C, Hohm A, Vandergrift LA, von Hoff K, Rutkowski S, Pietsch T, Warmuth-Metz M. MRI phenotype of RELA-fused pediatric supratentorial ependymoma. Clin Neuroradiol. 2019;29:595–604.
pubmed: 30027327
doi: 10.1007/s00062-018-0704-2
Fukuoka K, Kanemura Y, Shofuda T, et al. Significance of molecular classification of ependymomas: C11orf95-RELA fusion-negative supratentorial ependymomas are a heterogeneous group of tumors. Acta Neuropathol Commun. 2018;6:134.
pubmed: 30514397
pmcid: 6278135
doi: 10.1186/s40478-018-0630-1
Pagès M, Pajtler KW, Puget S, et al. Diagnostics of pediatric supratentorial RELA ependymomas: integration of information from histopathology, genetics, DNA methylation and imaging. Brain Pathol (Zurich, Switz). 2019;29:325–35.
doi: 10.1111/bpa.12664
Tauziède-Espariat A, Siegfried A, Nicaise Y, et al. 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. 2021;9:135.
pubmed: 34389065
pmcid: 8362233
doi: 10.1186/s40478-021-01238-y
Zschernack V, Jünger ST, Mynarek M, et al. Supratentorial ependymoma in childhood: more than just RELA or YAP. Acta Neuropathol (Berl). 2021;141:455. https://doi.org/10.1007/s00401-020-02260-5 .
doi: 10.1007/s00401-020-02260-5
pubmed: 33481105
Kupp R, Ruff L, Terranova S, et al. ZFTA translocations constitute ependymoma chromatin remodeling and transcription factors. Cancer Discov. 2021;11:2216–29.
pubmed: 33741711
pmcid: 8918067
doi: 10.1158/2159-8290.CD-20-1052
Pietsch T, Wohlers I, Goschzik T, Dreschmann V, Denkhaus D, Dörner E, Rahmann S, Klein-Hitpass L. Supratentorial ependymomas of childhood carry C11orf95-RELA fusions leading to pathological activation of the NF-κB signaling pathway. Acta Neuropathol (Berl). 2014;127:609–11.
pubmed: 24562983
doi: 10.1007/s00401-014-1264-4
Figarella-Branger D, Lechapt-Zalcman E, Tabouret E, et al. Supratentorial clear cell ependymomas with branching capillaries demonstrate characteristic clinicopathological features and pathological activation of nuclear factor-kappaB signaling. Neuro Oncol. 2016;18:919–27.
pubmed: 26984744
pmcid: 4896549
doi: 10.1093/neuonc/now025
Neumann JE, Spohn M, Obrecht D, et al. Molecular characterization of histopathological ependymoma variants. Acta Neuropathol (Berl). 2020;139:305–18.
pubmed: 31679042
doi: 10.1007/s00401-019-02090-0
Gessi M, Giagnacovo M, Modena P, et al. Role of immunohistochemistry in the identification of supratentorial C11ORF95-RELA fused ependymoma in routine neuropathology. Am J Surg Pathol. 2019;43:56–63.
pubmed: 29266023
doi: 10.1097/PAS.0000000000000979
Upadhyaya SA, Robinson GW, Onar-Thomas A, et al. Molecular grouping and outcomes of young children with newly diagnosed ependymoma treated on the multi-institutional SJYC07 trial. Neuro Oncol. 2019;21:1319. https://doi.org/10.1093/neuonc/noz069 .
doi: 10.1093/neuonc/noz069
pubmed: 30976811
pmcid: 6784269
Godfraind C, Kaczmarska JM, Kocak M, Dalton J, Wright KD, Sanford RA, Boop FA, Gajjar A, Merchant TE, Ellison DW. Distinct disease-risk groups in pediatric supratentorial and posterior fossa ependymomas. Acta Neuropathol (Berl). 2012;124:247–57.
pubmed: 22526017
doi: 10.1007/s00401-012-0981-9
Jünger ST, Andreiuolo F, Mynarek M, et al. CDKN2A deletion in supratentorial ependymoma with RELA alteration indicates a dismal prognosis: a retrospective analysis of the HIT ependymoma trial cohort. Acta Neuropathol (Berl). 2020;140:405. https://doi.org/10.1007/s00401-020-02169-z .
doi: 10.1007/s00401-020-02169-z
pubmed: 32514758
Shirahata M, Ono T, Stichel D, et al. Novel, improved grading system(s) for IDH-mutant astrocytic gliomas. Acta Neuropathol (Berl). 2018;136:153–66.
pubmed: 29687258
doi: 10.1007/s00401-018-1849-4
Jünger ST, Mynarek M, Wohlers I, et al. Improved risk-stratification for posterior fossa ependymoma of childhood considering clinical, histological and genetic features—a retrospective analysis of the HIT ependymoma trial cohort. Acta Neuropathol Commun. 2019;7:181.
pubmed: 31727173
pmcid: 6857225
doi: 10.1186/s40478-019-0820-5
Merchant TE. Current management of childhood ependymoma. Oncology (Williston Park N). 2002;16:629–42, 644; discussion 645–6, 648.
Pajtler KW, Wen J, Sill M, et al. Molecular heterogeneity and CXorf67 alterations in posterior fossa group A (PFA) ependymomas. Acta Neuropathol (Berl). 2018;136:211–26.
pubmed: 29909548
doi: 10.1007/s00401-018-1877-0
Baroni LV, Sundaresan L, Heled A, et al. Ultra high-risk PFA ependymoma is characterized by loss of chromosome 6q. Neuro Oncol. 2021;23:1360–70.
pubmed: 33580238
pmcid: 8328032
doi: 10.1093/neuonc/noab034
Ramaswamy V, Hielscher T, Mack SC, et al. Therapeutic impact of cytoreductive surgery and irradiation of posterior fossa ependymoma in the molecular era: a retrospective multicohort analysis. J Clin Oncol. 2016;34:2468–77.
pubmed: 27269943
pmcid: 4962737
doi: 10.1200/JCO.2015.65.7825
Thomas C, Thierfelder F, Träger M, et al. TERT promoter mutation and chromosome 6 loss define a high-risk subtype of ependymoma evolving from posterior fossa subependymoma. Acta Neuropathol (Berl). 2021;141:959–70.
pubmed: 33755803
doi: 10.1007/s00401-021-02300-8
Bayliss J, Mukherjee P, Lu C, et al. Lowered H3K27me3 and DNA hypomethylation define poorly prognostic pediatric posterior fossa ependymomas. Sci Transl Med. 2016;8:366ra161.
pubmed: 27881822
pmcid: 5123566
doi: 10.1126/scitranslmed.aah6904
Panwalkar P, Clark J, Ramaswamy V, et al. Immunohistochemical analysis of H3K27me3 demonstrates global reduction in group-A childhood posterior fossa ependymoma and is a powerful predictor of outcome. Acta Neuropathol (Berl). 2017;134:705–14.
pubmed: 28733933
doi: 10.1007/s00401-017-1752-4
Gessi M, Capper D, Sahm F, et al. Evidence of H3 K27M mutations in posterior fossa ependymomas. Acta Neuropathol (Berl). 2016;132:635–7.
pubmed: 27539613
doi: 10.1007/s00401-016-1608-3
Cavalli FMG, Hübner J-M, Sharma T, et al. Heterogeneity within the PF-EPN-B ependymoma subgroup. Acta Neuropathol (Berl). 2018;136:227–37.
pubmed: 30019219
doi: 10.1007/s00401-018-1888-x
Celano E, Salehani A, Malcolm JG, Reinertsen E, Hadjipanayis CG. Spinal cord ependymoma: a review of the literature and case series of ten patients. J Neurooncol. 2016;128:377–86.
pubmed: 27154165
pmcid: 5705940
doi: 10.1007/s11060-016-2135-8
Engelhard HH, Villano JL, Porter KR, Stewart AK, Barua M, Barker FG, Newton HB. Clinical presentation, histology, and treatment in 430 patients with primary tumors of the spinal cord, spinal meninges, or cauda equina. J Neurosurg Spine. 2010;13:67–77.
pubmed: 20594020
doi: 10.3171/2010.3.SPINE09430
Ghasemi DR, Sill M, Okonechnikov K, et al. MYCN amplification drives an aggressive form of spinal ependymoma. Acta Neuropathol (Berl). 2019;138:1075–89.
pubmed: 31414211
doi: 10.1007/s00401-019-02056-2
Scheil S, Brüderlein S, Eicker M, Herms J, Herold-Mende C, Steiner HH, Barth TF, Möller P. Low frequency of chromosomal imbalances in anaplastic ependymomas as detected by comparative genomic hybridization. Brain Pathol (Zurich, Switz). 2001;11:133–43.
doi: 10.1111/j.1750-3639.2001.tb00386.x
Swanson AA, Raghunathan A, Jenkins RB, Messing-Jünger M, Pietsch T, Clarke MJ, Kaufmann TJ, Giannini C. Spinal cord ependymomas with MYCN amplification show aggressive clinical behavior. J Neuropathol Exp Neurol. 2019;78:791–7.
pubmed: 31373367
doi: 10.1093/jnen/nlz064
Koeller KK, Rosenblum RS, Morrison AL. Neoplasms of the spinal cord and filum terminale: radiologic-pathologic correlation. Radiographics. 2000;20:1721–49.
pubmed: 11112826
doi: 10.1148/radiographics.20.6.g00nv151721
Ebert C, von Haken M, Meyer-Puttlitz B, Wiestler OD, Reifenberger G, Pietsch T, von Deimling A. Molecular genetic analysis of ependymal tumors. NF2 mutations and chromosome 22q loss occur preferentially in intramedullary spinal ependymomas. Am J Pathol. 1999;155:627–32.
pubmed: 10433955
pmcid: 1866851
doi: 10.1016/S0002-9440(10)65158-9
Benesch M, Frappaz D, Massimino M. Spinal cord ependymomas in children and adolescents. Childs Nerv Syst. 2012;28:2017–28.
pubmed: 22961356
doi: 10.1007/s00381-012-1908-4
Lee JC, Sharifai N, Dahiya S, et al. Clinicopathologic features of anaplastic myxopapillary ependymomas. Brain Pathol (Zurich, Switz). 2019;29:75–84.
doi: 10.1111/bpa.12673
Sonneland PR, Scheithauer BW, Onofrio BM. Myxopapillary ependymoma. A clinicopathologic and immunocytochemical study of 77 cases. Cancer. 1985;56:883–93.
pubmed: 4016681
doi: 10.1002/1097-0142(19850815)56:4<883::AID-CNCR2820560431>3.0.CO;2-6
Bockmayr M, Harnisch K, Pohl LC, et al. Comprehensive profiling of myxopapillary ependymomas identifies a distinct molecular subtype with relapsing disease. Neuro Oncol. 2022;24:1689–99.
pubmed: 35380708
pmcid: 9527524
doi: 10.1093/neuonc/noac088
Cervoni L, Celli P, Caruso R, Gagliardi FM, Cantore GP. [Neurinomas and ependymomas of the cauda equina. A review of the clinical characteristics]. Minerva Chir. 1997;52:629–33.
Bates JE, Choi G, Milano MT. Myxopapillary ependymoma: a SEER analysis of epidemiology and outcomes. J Neurooncol. 2016;129:251–8.
pubmed: 27306443
doi: 10.1007/s11060-016-2167-0
Yust Katz S, Cachia D, Kamiya-Matsuoka C, Olar A, Theeler B, Penas Prado M, Gilbert MR, Armstrong T. Ependymomas arising outside of the central nervous system: a case series and literature review. J Clin Neurosci. 2018;47:202–7.
pubmed: 29054328
doi: 10.1016/j.jocn.2017.10.026
Rudà R, Reifenberger G, Frappaz D, et al. EANO guidelines for the diagnosis and treatment of ependymal tumors. Neuro Oncol. 2018;20:445–56.
pubmed: 29194500
doi: 10.1093/neuonc/nox166
Abdallah A, Emel E, Gündüz HB, Sofuoğlu ÖE, Asiltürk M, Abdallah BG. Long-term surgical resection outcomes of pediatric myxopapillary ependymoma: experience of two centers and brief literature review. World Neurosurg. 2020;136:e245–61.
pubmed: 31899399
doi: 10.1016/j.wneu.2019.12.128
Bandopadhayay P, Silvera VM, Ciarlini PDSC, et al. Myxopapillary ependymomas in children: imaging, treatment and outcomes. J Neurooncol. 2016;126:165–74.
pubmed: 26468139
doi: 10.1007/s11060-015-1955-2
Weber DC, Wang Y, Miller R, et al. Long-term outcome of patients with spinal myxopapillary ependymoma: treatment results from the MD Anderson Cancer Center and institutions from the Rare Cancer Network. Neuro Oncol. 2015;17:588–95.
pubmed: 25301811
doi: 10.1093/neuonc/nou293
Raffeld M, Abdullaev Z, Pack SD, et al. High level MYCN amplification and distinct methylation signature define an aggressive subtype of spinal cord ependymoma. Acta Neuropathol Commun. 2020;8:101.
pubmed: 32641156
pmcid: 7346356
doi: 10.1186/s40478-020-00973-y
Adolph JE, Fleischhack G, Mikasch R, et al. Local and systemic therapy of recurrent ependymoma in children and adolescents: short- and long-term results of the E-HIT-REZ 2005 study. Neuro Oncol. 2020;23:1012. https://doi.org/10.1093/neuonc/noaa276 .
doi: 10.1093/neuonc/noaa276
pmcid: 8168820
Grill J, Le Deley M-C, Gambarelli D, et al. Postoperative chemotherapy without irradiation for ependymoma in children under 5 years of age: a multicenter trial of the French Society of Pediatric Oncology. J Clin Oncol. 2001;19:1288–96.
pubmed: 11230470
doi: 10.1200/JCO.2001.19.5.1288
Massimino M, Barretta F, Modena P, et al. Treatment and outcome of intracranial ependymoma after first relapse in the 2nd AIEOP protocol. Neuro Oncol. 2022;24:467–79.
pubmed: 34605902
doi: 10.1093/neuonc/noab230
Timmermann B, Kortmann R-D, Kühl J, Rutkowski S, Dieckmann K, Meisner C, Bamberg M. Role of radiotherapy in anaplastic ependymoma in children under age of 3 years: results of the prospective German brain tumor trials HIT-SKK 87 and 92. Radiother Oncol. 2005;77:278–85.
pubmed: 16300848
doi: 10.1016/j.radonc.2005.10.016
MacDonald SM, Safai S, Trofimov A, Wolfgang J, Fullerton B, Yeap BY, Bortfeld T, Tarbell NJ, Yock T. Proton radiotherapy for childhood ependymoma: initial clinical outcomes and dose comparisons. Int J Radiat Oncol Biol Phys. 2008;71:979–86.
pubmed: 18325681
doi: 10.1016/j.ijrobp.2007.11.065
Sato M, Gunther JR, Mahajan A, et al. Progression-free survival of children with localized ependymoma treated with intensity-modulated radiation therapy or proton-beam radiation therapy. Cancer. 2017;123:2570–8.
pubmed: 28267208
doi: 10.1002/cncr.30623
Peters S, Merta J, Schmidt L, et al. Evaluation of dose, volume, and outcome in children with localized, intracranial ependymoma treated with proton therapy within the prospective KiProReg study. Neuro Oncol. 2022;24:1193–202.
pubmed: 34964901
doi: 10.1093/neuonc/noab301
Indelicato DJ, Ioakeim-Ioannidou M, Bradley JA, Mailhot-Vega RB, Morris CG, Tarbell NJ, Yock T, MacDonald SM. Proton therapy for pediatric ependymoma: mature results from a bicentric study. Int J Radiat Oncol Biol Phys. 2021;110:815–20.
pubmed: 33508372
doi: 10.1016/j.ijrobp.2021.01.027
Ritzmann TA, Chapman RJ, Kilday J-P, et al. SIOP ependymoma I: final results, long-term follow-up, and molecular analysis of the trial cohort-A BIOMECA Consortium Study. Neuro Oncol. 2022;24:936–48.
pubmed: 35018471
pmcid: 9159435
doi: 10.1093/neuonc/noac012
Pfaff E, Kessler T, Balasubramanian GP, et al. Feasibility of real-time molecular profiling for patients with newly diagnosed glioblastoma without MGMT promoter hypermethylation-the NCT Neuro Master Match (N2M2) pilot study. Neuro Oncol. 2018;20:826–37.
pubmed: 29165638
doi: 10.1093/neuonc/nox216
Donovan LK, Delaidelli A, Joseph SK, et al. Locoregional delivery of CAR T cells to the cerebrospinal fluid for treatment of metastatic medulloblastoma and ependymoma. Nat Med. 2020;26:720–31.
pubmed: 32341580
pmcid: 8815773
doi: 10.1038/s41591-020-0827-2
Lötsch D, Kirchhofer D, Englinger B, et al. Targeting fibroblast growth factor receptors to combat aggressive ependymoma. Acta Neuropathol (Berl). 2021;142:339–60.
pubmed: 34046693
doi: 10.1007/s00401-021-02327-x
Han J, Yu M, Bai Y, et al. Elevated CXorf67 expression in PFA ependymomas suppresses DNA repair and sensitizes to PARP inhibitors. Cancer Cell. 2020;38:844–856.e7.
pubmed: 33186520
pmcid: 8455074
doi: 10.1016/j.ccell.2020.10.009
Feldman WB, Clark AJ, Safaee M, Ames CP, Parsa AT. Tumor control after surgery for spinal myxopapillary ependymomas: distinct outcomes in adults versus children: a systematic review. J Neurosurg Spine. 2013;19:471–6.
pubmed: 23971762
doi: 10.3171/2013.6.SPINE12927
Benesch M, Mynarek M, Witt H, et al. Newly diagnosed metastatic intracranial ependymoma in children: frequency, molecular characteristics, treatment, and outcome in the prospective HIT series. Oncologist. 2019;24:e921–9.
pubmed: 30850560
pmcid: 6738295
doi: 10.1634/theoncologist.2018-0489
Byer L, Kline CN, Coleman C, Allen IE, Whitaker E, Mueller S. A systematic review and meta-analysis of outcomes in pediatric, recurrent ependymoma. J Neurooncol. 2019;144:445–52.
pubmed: 31502040
doi: 10.1007/s11060-019-03255-3
Ritzmann TA, Rogers HA, Paine SML, Storer LCD, Jacques TS, Chapman RJ, Ellison D, Donson AM, Foreman NK, Grundy RG. A retrospective analysis of recurrent pediatric ependymoma reveals extremely poor survival and ineffectiveness of current treatments across central nervous system locations and molecular subgroups. Pediatr Blood Cancer. 2020;67:e28426.
pubmed: 32614133
doi: 10.1002/pbc.28426
Ritzmann TA, Kilday J-P, Grundy RG. Pediatric ependymomas: destined to recur? Neuro Oncol. 2021;23:874–6.
pubmed: 33728470
pmcid: 8168807
doi: 10.1093/neuonc/noab066