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Open Access 09-01-2025 | Meningioma | Review

Overall survival and progression-free survival in pediatric meningiomas: a systematic review and individual patient-level meta-analysis

Authors: Johannes Wach, Martin Vychopen, Alim Emre Basaran, Marcos Tatagiba, Roland Goldbrunner, Erdem Güresir

Abstract

Background

Pediatric meningiomas (PMs) are rare central nervous system tumors, accounting for 1–5% of all meningiomas, and differ from adult meningiomas in clinical, histopathological, and molecular features. Current guidelines primarily focus on adults, leaving a gap in evidence-based management for PMs. This study presents the largest meta-analysis of longitudinal individual patient data (IPD) to date, addressing progression-free survival (PFS) and overall survival (OS) in pediatric patients.

Methods

Data from 20 studies (2011–2023), including 1010 pediatric meningioma cases, were analyzed to assess PFS and OS stratified by WHO grade, NF1/NF2 status, extent of resection (EOR), and adjuvant radiotherapy. Longitudinal survival data were reconstructed from Kaplan–Meier curves using IPD extraction methods.

Results

PMs affect males and females nearly equally (52.1% vs. 47.9%). WHO grade 3 tumors had significantly shorter PFS (72.1 months) compared to grades 1 (209.8 months) and 2 (137.5 months) (p < 0.001). No significant OS difference between WHO grades 1 and 2 PMs were observed. NF1- and NF2-associated tumors showed shorter PFS (59.7 and 138.4 months) than sporadic cases (180.6 months) (p = 0.02). GTR significantly improved PFS (113.8 vs. 40.1 months, p < 0.001) and OS (602.9 vs. 173.8 months, p < 0.001). Radiotherapy enhanced PFS (72.5 vs. 23.8 months, p = 0.009) and OS (140.7 vs. 63.0 months, p = 0.002) in grade 3 tumors but not in WHO grade 2 PMs (p = 0.43).

Conclusions

This largest meta-analysis highlights the critical roles of GTR and adjuvant radiotherapy in improving outcomes for high-grade PMs and underscores the urgent need for pediatric-specific management guidelines based on robust longitudinal data.

Graphical Abstract

Available only for authorised users

Ostrom QT, Price M, Neff C et al (2023) CBTRUS statistical report: primary brain and other central nervous system tumors diagnosed in the United States in 2016–2020. Neuro Oncol 25(12 Suppl 2):iv1–iv99. https://doi.org/10.1093/neuonc/noad149CrossRefPubMedPubMedCentral

Sahm F, Schrimpf D, Stichel D et al (2017) DNA methylation-based classification and grading system for meningioma: a multicentre, retrospective analysis. Lancet Oncol 18(5):682–694. https://doi.org/10.1016/S1470-2045(17)30155-9CrossRefPubMed

Nassiri F, Liu J, Patil V et al (2021) A clinically applicable integrative molecular classification of meningiomas. Nature 597(7874):119–125. https://doi.org/10.1038/s41586-021-03850-3CrossRefPubMedPubMedCentral

Tauziède-Espariat A, Pfister SM, Mawrin C, Sahm F (2023) Pediatric meningiomas: A literature review and diagnostic update. Neurooncol Adv 5(Suppl 1):i105–i111. https://doi.org/10.1093/noajnl/vdac165CrossRefPubMedPubMedCentral

Ostrom QT, Gittleman H, Fulop J et al (2015) CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2008–2012. Neuro Oncol 17(Suppl 4):iv1–iv62. https://doi.org/10.1093/neuonc/nov189CrossRefPubMedPubMedCentral

Kotecha RS, Pascoe EM, Rushing EJ et al (2011) Meningiomas in children and adolescents: a meta-analysis of individual patient data. Lancet Oncol 12(13):1229–1239. https://doi.org/10.1016/S1470-2045(11)70275-3CrossRefPubMed

Goldbrunner R, Stavrinou P, Jenkinson MD et al (2021) EANO guideline on the diagnosis and management of meningiomas. Neuro Oncol 23(11):1821–1834. https://doi.org/10.1093/neuonc/noab150CrossRefPubMedPubMedCentral

Kirches E, Sahm F, Korshunov A et al (2021) Molecular profiling of pediatric meningiomas shows tumor characteristics distinct from adult meningiomas. Acta Neuropathol 142(5):873–886. https://doi.org/10.1007/s00401-021-02351-xCrossRefPubMedPubMedCentral

Battu S, Kumar A, Pathak P et al (2018) Clinicopathological and molecular characteristics of pediatric meningiomas. Neuropathology 38(1):22–33. https://doi.org/10.1111/neup.12426CrossRefPubMed

10.

Fountain DM, Smith MJ, O’Leary C et al (2021) The spatial phenotype of genotypically distinct meningiomas demonstrate potential implications of the embryology of the meninges. Oncogene 40(5):875–884. https://doi.org/10.1038/s41388-020-01568-6CrossRefPubMed

11.

Toland A, McNulty SN, Pekmezci M et al (2020) Pediatric meningioma: a clinicopathologic and molecular study with potential grading implications. Brain Pathol 30(6):1134–1143. https://doi.org/10.1111/bpa.12884CrossRefPubMedPubMedCentral

12.

Stewart LA, Clarke M, Rovers M et al (2015) Preferred reporting items for systematic review and meta-analyses of individual participant data: the PRISMA-IPD statement. JAMA 313(16):1657–1665. https://doi.org/10.1001/jama.2015.36564CrossRefPubMed

13.

Ma LL, Wang YY, Yang ZH, Huang D, Weng H, Zeng XT (2020) Methodological quality (risk of bias) assessment tools for primary and secondary medical studies: what are they and which is better? Mil Med Res 7(1):7. https://doi.org/10.1186/s40779-020-00238-8CrossRefPubMedPubMedCentral

14.

Jagtiani P, Karabacak M, Le C, Bahadir Z, Morgenstern P, Margetis K (2024) Comprehensive assessment of atypical and anaplastic pediatric meningiomas utilizing national cancer database: a retrospective cohort study. Childs Nerv Syst 40(8):2345–2357. https://doi.org/10.1007/s00381-024-06431-7CrossRefPubMed

15.

Liu N, Zhou Y, Lee JJ (2021) IPDfromKM: reconstruct individual patient data from published Kaplan-Meier survival curves. BMC Med Res Methodol 21(1):111CrossRefPubMedPubMedCentral

16.

Rakap S, Rakap S, Evran D, Cig O (2016) Comparative evaluation of the reliability and validity of three data extraction programs: UnGraph, GraphClick, and DigitizeIt. Comput Hum Behav 55:159–166. https://doi.org/10.1016/j.chb.2015.09.008CrossRef

17.

Gader G, Slimane A, Sliti F, Badri M, Zammel I (2024) Paediatric rhabdoid meningioma: clinical and therapeutic features findings - case series of 3 patients. Case Rep Pediatr Neurosurg 59(1):20–26. https://doi.org/10.1159/000535715CrossRef

18.

García-Marqueta M, Vázquez M, Krcek R et al (2023) Quality of life, clinical, and patient-reported outcomes after pencil beam scanning proton therapy delivered for intracranial grade WHO 1–2 meningioma in children and adolescents. Cancers (Basel) 15(18):4447. https://doi.org/10.3390/cancers15184447CrossRefPubMed

19.

Chen Y, Tian S, Wang J et al (2023) Lateral ventricle meningiomas in children: clinicopathological and neuroradiological features. Childs Nerv Syst 39(1):151–158. https://doi.org/10.1007/s00381-022-05680-8CrossRefPubMed

20.

Opoku I, Yang L, Sun P et al (2022) Pediatric Cerebral meningioma: a single-center study with 10 children not associated with neurofibromatosis type 2 and literature review. Pediatr Neurosurg 57(6):422–433. https://doi.org/10.1159/000526541CrossRefPubMed

21.

Santana-González A, Pérez-Ramírez M, Ramírez-Reyes G et al (2021) Management experience of pediatric meningioma in a Mexican reference center. Ann Diagn Pathol 55:151811. https://doi.org/10.1016/j.anndiagpath.2021.151811CrossRefPubMed

22.

Liu J, Zhao K, Wang J, Shu K (2021) Clinical features and long-term outcomes of pediatric meningiomas. Childs Nerv Syst 37(10):3041–3047. https://doi.org/10.1007/s00381-021-05296-4CrossRefPubMed

23.

Fouda MA, Day EL, Zurakowski D et al (2021) Predictors of progression in radiation-induced versus nonradiation-induced pediatric meningiomas: a large single-institution surgical experience. J Neurosurg Pediatr 28(2):160–166. https://doi.org/10.3171/2021.1.PEDS20819CrossRefPubMed

24.

He W, Liu Z, Teng H et al (2020) Pediatric meningiomas: 10-year experience with 39 patients. J Neurooncol 149(3):543–553. https://doi.org/10.1007/s11060-020-03649-8CrossRefPubMed

25.

Amirjamshidi A, Khalatbari MR, Abbasioun K, Ghasemi B (2019) Sylvian fissure meningioma in childhood: report of 3 cases and review of the literature. Pediatr Neurosurg 54(4):270–276. https://doi.org/10.1159/000500765CrossRefPubMed

26.

Tauziede-Espariat A, Parfait B, Besnard A et al (2018) Loss of SMARCE1 expression is a specific diagnostic marker of clear cell meningioma: a comprehensive immunophenotypical and molecular analysis. Brain Pathol 28(4):466–474. https://doi.org/10.1111/bpa.12524CrossRefPubMed

27.

Liu H, Luo W, Li J, Yang J, Xu Y (2017) Pediatric infratentorial meningiomas: a series of 19 cases and review of the literature. Childs Nerv Syst 33(5):777–786. https://doi.org/10.1007/s00381-017-3362-9CrossRefPubMed

28.

Dash C, Pasricha R, Gurjar H, Singh PK, Sharma BS (2016) Pediatric intraventricular meningioma: a series of six cases. J Pediatr Neurosci 11(3):193–196. https://doi.org/10.4103/1817-1745.193356CrossRefPubMedPubMedCentral

29.

Donovan DJ, Thavapalan V (2016) Pediatric meningeal tumors of the sylvian fissure region without dural attachment: a series of three patients and review of the literature. Surg J (N Y). https://doi.org/10.1055/s-0036-1584166CrossRefPubMed

30.

Burkhardt JK, Neidert MC, Grotzer MA, Krayenbühl N, Bozinov O (2013) Surgical resection of pediatric skull base meningiomas. Childs Nerv Syst 29(1):83–87. https://doi.org/10.1007/s00381-012-1906-6CrossRefPubMed

31.

Wang XQ, Jiang CC, Zhao L, Gong Y, Hu J, Chen H (2012) Clinical features and treatment of World Health Organization grade II and III meningiomas in childhood: report of 23 cases. J Neurosurg Pediatr 10(5):423–433. https://doi.org/10.3171/2012.7.PEDS12179CrossRefPubMed

32.

Santos MV, Furlanetti L, Valera ET, Brassesco MS, Tone LG, de Oliveira RS (2012) Pediatric meningiomas: a single-center experience with 15 consecutive cases and review of the literature. Childs Nerv Syst 28(11):1887–1896. https://doi.org/10.1007/s00381-012-1823-8CrossRefPubMed

33.

Jaiswal S, Vij M, Mehrotra A, Jaiswal AK, Srivastava AK, Behari S (2011) A clinicopathological and neuroradiological study of paediatric meningioma from a single centre. J Clin Neurosci 18(8):1084–1089. https://doi.org/10.1016/j.jocn.2010.11.036CrossRefPubMed

34.

Arivazhagan A, Devi BI, Kolluri SVR et al (2008) Pediatric intracranial meningiomas – do they differ from their counterparts in adults? Pediatr Neurosurg 44:43–48CrossRefPubMed

35.

Dolecek TA, Dressler EVM, Thakkar JP et al (2015) Epidemiology of meningiomas post-public law 107–206: the benign brain tumor cancer registries amendment act. Cancer 121(14):2400–2410CrossRefPubMed

36.

Champeaux-Depond C, Weller J, Froelich S, Resche-Rigon M (2021) A nationwide population-based study on overall survival after meningioma surgery. Cancer Epidemiol 70:101875CrossRefPubMed

37.

Louis DN, Perry A, Wesseling P et al (2021) The 2021 WHO classification of tumors of the central nervous system: a summary. Neuro Oncol 23(8):1231–1251. https://doi.org/10.1093/neuonc/noab106CrossRefPubMedPubMedCentral

38.

Kirches E, Sahm F, Korshunov A et al (2021) Molecular profiling of pedi- atric meningiomas shows tumor characteristics distinct from adult meningiomas. Acta Neuropathol 142(5):873–886CrossRefPubMedPubMedCentral

39.

Perry A, Giannini C, Raghavan R et al (2001) Aggressive phenotypic and genotypic features in pediatric and NF2-associated meningiomas: a clinicopathologic study of 53 cases. J Neuropathol Exp Neurol 60(10):994–1003CrossRefPubMed

40.

Sievers P, Chiang J, Schrimpf D et al (2020) YAP1-fusions in pediatric NF2- wildtype meningioma. Acta Neuropathol 139(1):215–218CrossRefPubMed

41.

Tamura R, Yamanobe Y, Fujioka M et al (2024) Phase I/II study of a vascular endothelial growth factor receptor vaccine in patients with NF2-related schwannomatosis. J Clin Oncol 42(21):2578–2587. https://doi.org/10.1200/JCO.23.02376CrossRefPubMed

42.

Szychot E, Goodden J, Whitfield G, Curry S (2020) Children’s cancer and leukaemia group (CCLG): review and guidelines for the management of meningioma in children, teenagers and young adults. Br J Neurosurg 34(2):142–153. https://doi.org/10.1080/02688697.2020.1726286CrossRefPubMed

43.

Wach J, Basaran AE, Arlt F et al (2023) CDKN2A/B deletions are strongly associated with meningioma progression: a meta-analysis of individual patient data. Acta Neuropathol Commun 11(1):189. https://doi.org/10.1186/s40478-023-01690-yCrossRefPubMedPubMedCentral

44.

Mirian C, Duun-Henriksen AK, Juratli T et al (2020) Poor prognosis associated with TERT gene alterations in meningioma is independent of the WHO classification: an individual patient data meta-analysis. J Neurol Neurosurg Psychiatry 91(4):378–387. https://doi.org/10.1136/jnnp-2019-322257CrossRefPubMed

45.

Vasan V, Dullea JT, Devarajan A et al (2023) NF2 mutations are associated with resistance to radiation therapy for grade 2 and grade 3 recurrent meningiomas. J Neurooncol 161(2):309–316. https://doi.org/10.1007/s11060-022-04197-zCrossRefPubMed

46.

Girvigian MR, Chen JC, Rahimian J, Miller MJ, Tome M (2008) Comparison of early complications for patients with convexity and parasagittal meningiomas treated with either stereotactic radiosurgery or fractionated stereotactic radiotherapy. Neurosurgery 62(5 Suppl):A19–A27. https://doi.org/10.1227/01.neu.0000325933.34154.cb. (discussion A27-8)CrossRefPubMed

47.

Jenkinson MD, Javadpour M, Haylock BJ et al (2015) The ROAM/EORTC-1308 trial: radiation versus observation following surgical resection of atypical meningioma: study protocol for a randomised controlled trial. Trials 16:519. https://doi.org/10.1186/s13063-015-1040-3CrossRefPubMedPubMedCentral

48.

Rogers CL, Won M, Vogelbaum MA et al (2020) High-risk meningioma: initial outcomes from NRG oncology/RTOG 0539. Int J Radiat Oncol Biol Phys 106(4):790–799. https://doi.org/10.1016/j.ijrobp.2019.11.028CrossRefPubMed

49.

Wang JZ, Patil V, Landry AP et al (2024) Molecular classification to refine surgical and radiotherapeutic decision-making in meningioma. Nat Med. https://doi.org/10.1038/s41591-024-03167-4CrossRefPubMedPubMedCentral

50.

Kallio M, Sankila R, Hakulinen T, Jääskeläinen J (1992) Factors affecting operative and excess long-term mortality in 935 patients with intracranial meningioma. Neurosurgery 31(1):2–12. https://doi.org/10.1227/00006123-199207000-00002CrossRefPubMed

Title: Overall survival and progression-free survival in pediatric meningiomas: a systematic review and individual patient-level meta-analysis
Authors: Johannes Wach
Martin Vychopen
Alim Emre Basaran
Marcos Tatagiba
Roland Goldbrunner
Erdem Güresir
Publication date: 09-01-2025
Publisher: Springer US
Keywords: Meningioma
Meningioma
Neurofibromatosis
Neurofibromatosis
Radiotherapy
Published in: Journal of Neuro-Oncology
Print ISSN: 0167-594X
Electronic ISSN: 1573-7373
DOI: https://doi.org/10.1007/s11060-024-04917-7

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