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Semin Neurol 2023; 43(06): 909-928
DOI: 10.1055/s-0043-1776775
Review Article

Brain Tumors in Adolescents and Young Adults: A Review

Julien Rousseau
1   Division of Neurology, Department of Medicine, Universite de Montreal, Montreal, Quebec, Canada
,
Julie Bennett
2   Division of Haematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada
3   Canadian AYA Neuro-Oncology Network (CANON), Toronto, Ontario, Canada
,
Mary Jane Lim-Fat
3   Canadian AYA Neuro-Oncology Network (CANON), Toronto, Ontario, Canada
4   Division of Neurology, Department of Medicine, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ontario, Canada
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Abstract

Brain tumors account for the majority of cancer-related deaths in adolescents and young adults (AYAs), defined as individuals aged 15 to 39. AYAs constitute a distinct population in which both pediatric- and adult-type central nervous system (CNS) tumors can be observed. Clinical manifestations vary depending on tumor location and often include headaches, seizures, focal neurological deficits, and signs of increased intracranial pressure. With the publication of the updated World Health Organization CNS tumor classification in 2021, diagnoses have been redefined to emphasize key molecular alterations. Gliomas represent the majority of malignant brain tumors in this age group. Glioneuronal and neuronal tumors are associated with longstanding refractory epilepsy. The classification of ependymomas and medulloblastomas has been refined, enabling better identification of low-risk tumors that could benefit from treatment de-escalation strategies. Owing to their midline location, germ cell tumors often present with oculomotor and visual alterations as well as endocrinopathies. The management of CNS tumors in AYA is often extrapolated from pediatric and adult guidelines, and generally consists of a combination of surgical resection, radiation therapy, and systemic therapy. Ongoing research is investigating multiple agents targeting molecular alterations, including isocitrate dehydrogenase inhibitors, SHH pathway inhibitors, and BRAF inhibitors. AYA patients with CNS tumors should be managed by multidisciplinary teams and counselled regarding fertility preservation, psychosocial comorbidities, and risks of long-term comorbidities. There is a need for further efforts to design clinical trials targeting CNS tumors in the AYA population.

Keywords

brain neoplasms - glioma - adolescents and young adults


Publication History

Article published online:
10 November 2023

© 2023. Thieme. All rights reserved.

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  • References

  • 1 Ostrom QT, Cioffi G, Waite K, Kruchko C, Barnholtz-Sloan JS. CBTRUS statistical report: primary brain and other central nervous system tumors diagnosed in the United States in 2014–2018. Neuro-oncol 2021; 23 (12, suppl 2): iii1 iii105.
    CrossrefPubMedGoogle Scholar
  • 2 Lim-Fat MJ, Macdonald M, Lapointe S. et al. Molecular testing for adolescent and young adult central nervous system tumors: a Canadian guideline. Front Oncol 2022; 12: 960509
    CrossrefPubMedGoogle Scholar
  • 3 Board WHOCoTE, World Health O, International Agency for Research on C.. WHO classification of tumours: central nervous system tumours. 5th edition. ed. Lyon: International Agency for Research on Cancer; 2021
    Google Scholar
  • 4 Louis DN, Perry A, Wesseling P. et al. The 2021 WHO classification of tumors of the central nervous system: a summary. Neuro-oncol 2021; 23 (08) 1231-1251
    CrossrefPubMedGoogle Scholar
  • 5 Hartmann C, Meyer J, Balss J. et al. Type and frequency of IDH1 and IDH2 mutations are related to astrocytic and oligodendroglial differentiation and age: a study of 1,010 diffuse gliomas. Acta Neuropathol 2009; 118 (04) 469-474
    CrossrefPubMedGoogle Scholar
  • 6 DeWitt JC, Jordan JT, Frosch MP. et al. Cost-effectiveness of IDH testing in diffuse gliomas according to the 2016 WHO classification of tumors of the central nervous system recommendations. Neuro-oncol 2017; 19 (12) 1640-1650
    CrossrefPubMedGoogle Scholar
  • 7 van den Bent MJ, Afra D, de Witte O. et al; EORTC Radiotherapy and Brain Tumor Groups and the UK Medical Research Council. Long-term efficacy of early versus delayed radiotherapy for low-grade astrocytoma and oligodendroglioma in adults: the EORTC 22845 randomised trial. Lancet 2005; 366 (9490) 985-990
    CrossrefPubMedGoogle Scholar
  • 8 Pignatti F, van den Bent M, Curran D. et al; European Organization for Research and Treatment of Cancer Brain Tumor Cooperative Group, European Organization for Research and Treatment of Cancer Radiotherapy Cooperative Group. Prognostic factors for survival in adult patients with cerebral low-grade glioma. J Clin Oncol 2002; 20 (08) 2076-2084
    CrossrefPubMedGoogle Scholar
  • 9 Study of Vorasidenib (AG-881) in Participants with Residual or Recurrent Grade 2 Glioma with an IDH1 or IDH2 Mutation (INDIGO). ClinicalTrials.gov. 2019 . Accessed October 27, 2023 at: https://clinicaltrials.gov/ct2/show/NCT04164901
    PubMedGoogle Scholar
  • 10 Pediatric central nervous system cancers. National Comprehensive Cancer Network. 2022 [updated October 31, 2022 ]. Accessed October 27, 2023 at: https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1509
    PubMedGoogle Scholar
  • 11 Mohile NA, Messersmith H, Gatson NT. et al. Therapy for diffuse astrocytic and oligodendroglial tumors in adults: ASCO-SNO guideline. J Clin Oncol 2022; 40 (04) 403-426
    CrossrefPubMedGoogle Scholar
  • 12 Shaw EG, Wang M, Coons SW. et al. Randomized trial of radiation therapy plus procarbazine, lomustine, and vincristine chemotherapy for supratentorial adult low-grade glioma: initial results of RTOG 9802. J Clin Oncol 2012; 30 (25) 3065-3070
    CrossrefPubMedGoogle Scholar
  • 13 van den Bent MJ, Brandes AA, Taphoorn MJ. et al. Adjuvant procarbazine, lomustine, and vincristine chemotherapy in newly diagnosed anaplastic oligodendroglioma: long-term follow-up of EORTC brain tumor group study 26951. J Clin Oncol 2013; 31 (03) 344-350
    CrossrefPubMedGoogle Scholar
  • 14 Berger TR, Wen PY, Lang-Orsini M, Chukwueke UN. World Health Organization 2021 classification of central nervous system tumors and implications for therapy for adult-type gliomas: a review. JAMA Oncol 2022; 8 (10) 1493-1501
    CrossrefPubMedGoogle Scholar
  • 15 Patel SH, Poisson LM, Brat DJ. et al. T2-FLAIR mismatch, an imaging biomarker for IDH and 1p/19q status in lower-grade gliomas: a TCGA/TCIA project. Clin Cancer Res 2017; 23 (20) 6078-6085
    CrossrefPubMedGoogle Scholar
  • 16 Johnson DR, Kaufmann TJ, Patel SH, Chi AS, Snuderl M, Jain R. There is an exception to every rule-T2-FLAIR mismatch sign in gliomas. Neuroradiology 2019; 61 (02) 225-227
    CrossrefPubMedGoogle Scholar
  • 17 van den Bent MJ, Baumert B, Erridge SC. et al. Interim results from the CATNON trial (EORTC study 26053–22054) of treatment with concurrent and adjuvant temozolomide for 1p/19q non-co-deleted anaplastic glioma: a phase 3, randomised, open-label intergroup study. Lancet 2017; 390 (10103): 1645-1653
    CrossrefPubMedGoogle Scholar
  • 18 Wen PY, Weller M, Lee EQ. et al. Glioblastoma in adults: a Society for Neuro-Oncology (SNO) and European Society of Neuro-Oncology (EANO) consensus review on current management and future directions. Neuro-oncol 2020; 22 (08) 1073-1113
    CrossrefPubMedGoogle Scholar
  • 19 Stupp R, Mason WP, van den Bent MJ. et al; European Organisation for Research and Treatment of Cancer Brain Tumor and Radiotherapy Groups, National Cancer Institute of Canada Clinical Trials Group. Radiotherapy plus concomitant and adjuvant temozolomide for glioblastoma. N Engl J Med 2005; 352 (10) 987-996
    CrossrefPubMedGoogle Scholar
  • 20 Stupp R, Taillibert S, Kanner A. et al. Effect of tumor-treating fields plus maintenance temozolomide vs maintenance temozolomide alone on survival in patients with glioblastoma: a randomized clinical trial. JAMA 2017; 318 (23) 2306-2316
    CrossrefPubMedGoogle Scholar
  • 21 Ostrom QT, Price M, Ryan K. et al. CBTRUS statistical report: pediatric brain tumor foundation childhood and adolescent primary brain and other central nervous system tumors diagnosed in the United States in 2014–2018. Neuro-oncol 2022; 24 (suppl 3): iii1-iii38
    CrossrefPubMedGoogle Scholar
  • 22 Bennett J, Erker C, Lafay-Cousin L. et al. Canadian pediatric neuro-oncology standards of practice. Front Oncol 2020; 10: 593192
    CrossrefPubMedGoogle Scholar
  • 23 Gnekow AK, Kandels D, Tilburg CV. et al. SIOP-E-BTG and GPOH guidelines for diagnosis and treatment of children and adolescents with low grade glioma. Klin Padiatr 2019; 231 (03) 107-135
    Article in Thieme ConnectPubMedGoogle Scholar
  • 24 Krishnatry R, Zhukova N, Guerreiro Stucklin AS. et al. Clinical and treatment factors determining long-term outcomes for adult survivors of childhood low-grade glioma: a population-based study. Cancer 2016; 122 (08) 1261-1269
    CrossrefPubMedGoogle Scholar
  • 25 Lassaletta A, Scheinemann K, Zelcer SM. et al. Phase II weekly vinblastine for chemotherapy-naïve children with progressive low-grade glioma: a Canadian Pediatric Brain Tumor Consortium study. J Clin Oncol 2016; 34 (29) 3537-3543
    CrossrefPubMedGoogle Scholar
  • 26 Packer RJ, Lange B, Ater J. et al. Carboplatin and vincristine for recurrent and newly diagnosed low-grade gliomas of childhood. J Clin Oncol 1993; 11 (05) 850-856
    CrossrefPubMedGoogle Scholar
  • 27 Rosca L, Robert-Boire V, Delisle JF, Samson Y, Perreault S. Carboplatin and vincristine neurotoxicity in the treatment of pediatric low-grade gliomas. Pediatr Blood Cancer 2018; 65 (11) e27351
    CrossrefPubMedGoogle Scholar
  • 28 Ryall S, Zapotocky M, Fukuoka K. et al. Integrated molecular and clinical analysis of 1,000 pediatric low-grade gliomas. Cancer Cell 2020; 37 (04) 569-583.e5
    CrossrefPubMedGoogle Scholar
  • 29 Banerjee A, Jakacki RI, Onar-Thomas A. et al. A phase I trial of the MEK inhibitor selumetinib (AZD6244) in pediatric patients with recurrent or refractory low-grade glioma: a Pediatric Brain Tumor Consortium (PBTC) study. Neuro-oncol 2017; 19 (08) 1135-1144
    CrossrefPubMedGoogle Scholar
  • 30 Fangusaro J, Onar-Thomas A, Young Poussaint T. et al. Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial. Lancet Oncol 2019; 20 (07) 1011-1022
    CrossrefPubMedGoogle Scholar
  • 31 Fangusaro J, Onar-Thomas A, Poussaint TY. et al. A phase II trial of selumetinib in children with recurrent optic pathway and hypothalamic low-grade glioma without NF1: a Pediatric Brain Tumor Consortium study. Neuro-oncol 2021; 23 (10) 1777-1788
    CrossrefPubMedGoogle Scholar
  • 32 Andrews LJ, Thornton ZA, Saincher SS. et al. Prevalence of BRAFV600 in glioma and use of BRAF Inhibitors in patients with BRAFV600 mutation-positive glioma: systematic review. Neuro-oncol 2022; 24 (04) 528-540
    CrossrefPubMedGoogle Scholar
  • 33 Wen PY, Stein A, van den Bent M. et al. Dabrafenib plus trametinib in patients with BRAFV600E-mutant low-grade and high-grade glioma (ROAR): a multicentre, open-label, single-arm, phase 2, basket trial. Lancet Oncol 2022; 23 (01) 53-64
    CrossrefPubMedGoogle Scholar
  • 34 Phase II Pediatric Study with Dabrafenib in Combination with Trametinib in Patients with HGG and LGG. ClinicalTrials.gov. 2022 . Accessed October 21, 2022 at: https://clinicaltrials.gov/ct2/show/NCT02684058
    PubMedGoogle Scholar
  • 35 A Study of the Drugs Selumetinib Versus Carboplatin/Vincristine in Patients with Neurofibromatosis and Low-Grade Glioma. ClinicalTrials.gov. 2022 . Accessed October 21, 2022 at: https://clinicaltrials.gov/ct2/show/NCT03871257
    PubMedGoogle Scholar
  • 36 Trametinib for Pediatric Neuro-oncology Patients with Refractory Tumor and Activation of the MAPK/ERK Pathway. ClinicalTrials.gov. 2022 . Accessed October 21, 2022 at: https://clinicaltrials.gov/ct2/show/NCT03363217
    PubMedGoogle Scholar
  • 37 Gregory TA, Chumbley LB, Henson JW, Theeler BJ. Adult pilocytic astrocytoma in the molecular era: a comprehensive review. CNS Oncol 2021; 10 (01) CNS68
    CrossrefPubMedGoogle Scholar
  • 38 Salles D, Santino SF, Ribeiro DA, Malinverni ACM, Stávale JN. The involvement of the MAPK pathway in pilocytic astrocytomas. Pathol Res Pract 2022; 232: 153821
    CrossrefPubMedGoogle Scholar
  • 39 Milde T, Rodriguez FJ, Barnholtz-Sloan JS, Patil N, Eberhart CG, Gutmann DH. Reimagining pilocytic astrocytomas in the context of pediatric low-grade gliomas. Neuro-oncol 2021; 23 (10) 1634-1646
    CrossrefPubMedGoogle Scholar
  • 40 Theeler BJ, Ellezam B, Sadighi ZS. et al. Adult pilocytic astrocytomas: clinical features and molecular analysis. Neuro-oncol 2014; 16 (06) 841-847
    CrossrefPubMedGoogle Scholar
  • 41 Lee KJ, Marchan E, Peterson J. et al. Management and survival of adult patients with pilocytic astrocytoma in the national cancer database. World Neurosurg 2018; 112: e881-e887
    CrossrefPubMedGoogle Scholar
  • 42 Koeller KK, Rushing EJ. From the archives of the AFIP: pilocytic astrocytoma: radiologic-pathologic correlation. Radiographics 2004; 24 (06) 1693-1708
    CrossrefPubMedGoogle Scholar
  • 43 Gaudino S, Martucci M, Russo R. et al. MR imaging of brain pilocytic astrocytoma: beyond the stereotype of benign astrocytoma. Childs Nerv Syst 2017; 33 (01) 35-54
    CrossrefPubMedGoogle Scholar
  • 44 Bornhorst M, Frappaz D, Packer RJ. Pilocytic astrocytomas. Handb Clin Neurol 2016; 134: 329-344
    CrossrefPubMedGoogle Scholar
  • 45 Bond KM, Hughes JD, Porter AL, Orina J, Fang S, Parney IF. Adult pilocytic astrocytoma: an institutional series and systematic literature review for extent of resection and recurrence. World Neurosurg 2018; 110: 276-283
    CrossrefPubMedGoogle Scholar
  • 46 Dodgshun AJ, Maixner WJ, Hansford JR, Sullivan MJ. Low rates of recurrence and slow progression of pediatric pilocytic astrocytoma after gross-total resection: justification for reducing surveillance imaging. J Neurosurg Pediatr 2016; 17 (05) 569-572
    CrossrefPubMedGoogle Scholar
  • 47 Nelson AJ, Zakaria R, Jenkinson MD, Brodbelt AR. Extent of resection predicts risk of progression in adult pilocytic astrocytoma. Br J Neurosurg 2019; 33 (03) 343-347
    CrossrefPubMedGoogle Scholar
  • 48 Ogiwara H, Bowman RM, Tomita T. Long-term follow-up of pediatric benign cerebellar astrocytomas. Neurosurgery 2012; 70 (01) 40-47 , discussion 47–48
    CrossrefPubMedGoogle Scholar
  • 49 Johnson DR, Brown PD, Galanis E, Hammack JE. Pilocytic astrocytoma survival in adults: analysis of the Surveillance, Epidemiology, and End Results Program of the National Cancer Institute. J Neurooncol 2012; 108 (01) 187-193
    CrossrefPubMedGoogle Scholar
  • 50 Parsa CF, Givrad S. Juvenile pilocytic astrocytomas do not undergo spontaneous malignant transformation: grounds for designation as hamartomas. Br J Ophthalmol 2008; 92 (01) 40-46
    CrossrefPubMedGoogle Scholar
  • 51 McAuley E, Brophy H, Hayden J. et al. The benefit of surveillance imaging for paediatric cerebellar pilocytic astrocytoma. Childs Nerv Syst 2019; 35 (05) 801-805
    CrossrefPubMedGoogle Scholar
  • 52 Bender K, Perez E, Chirica M. et al. High-grade astrocytoma with piloid features (HGAP): the Charité experience with a new central nervous system tumor entity. J Neurooncol 2021; 153 (01) 109-120
    CrossrefPubMedGoogle Scholar
  • 53 Reinhardt A, Stichel D, Schrimpf D. et al. Anaplastic astrocytoma with piloid features, a novel molecular class of IDH wildtype glioma with recurrent MAPK pathway, CDKN2A/B and ATRX alterations. Acta Neuropathol 2018; 136 (02) 273-291
    CrossrefPubMedGoogle Scholar
  • 54 Rudà R, Capper D, Waldman AD. et al. EANO - EURACAN - SNO guidelines on circumscribed astrocytic gliomas, glioneuronal, and neuronal tumors. Neuro-oncol 2022; 24 (12) 2015-2034
    CrossrefPubMedGoogle Scholar
  • 55 Perkins SM, Mitra N, Fei W, Shinohara ET. Patterns of care and outcomes of patients with pleomorphic xanthoastrocytoma: a SEER analysis. J Neurooncol 2012; 110 (01) 99-104
    CrossrefPubMedGoogle Scholar
  • 56 Ida CM, Rodriguez FJ, Burger PC. et al. Pleomorphic xanthoastrocytoma: natural history and long-term follow-up. Brain Pathol 2015; 25 (05) 575-586
    CrossrefPubMedGoogle Scholar
  • 57 Kaley T, Touat M, Subbiah V. et al. BRAF inhibition in BRAF V600-mutant gliomas: results from the VE-BASKET study. J Clin Oncol 2018; 36 (35) 3477-3484
    CrossrefPubMedGoogle Scholar
  • 58 Kata K, Rodriguez-Quintero JC, Arevalo OD. et al. BRAF/MEK dual inhibitors therapy in progressive and anaplastic pleomorphic xanthoastrocytoma: case series and literature review. J Natl Compr Canc Netw 2022; 20 (11) 1193-1202.e6
    CrossrefPubMedGoogle Scholar
  • 59 Chen W, Soon YY, Pratiseyo PD. et al. Central nervous system neuroepithelial tumors with MN1-alteration: an individual patient data meta-analysis of 73 cases. Brain Tumor Pathol 2020; 37 (04) 145-153
    CrossrefPubMedGoogle Scholar
  • 60 Janz C, Buhl R. Astroblastoma: report of two cases with unexpected clinical behavior and review of the literature. Clin Neurol Neurosurg 2014; 125: 114-124
    CrossrefPubMedGoogle Scholar
  • 61 Chiang J, Harreld JH, Tinkle CL. et al. A single-center study of the clinicopathologic correlates of gliomas with a MYB or MYBL1 alteration. Acta Neuropathol 2019; 138 (06) 1091-1092
    CrossrefPubMedGoogle Scholar
  • 62 Ellison DW, Hawkins C, Jones DTW. et al. cIMPACT-NOW update 4: diffuse gliomas characterized by MYB, MYBL1, or FGFR1 alterations or BRAFV600E mutation. Acta Neuropathol 2019; 137 (04) 683-687
    CrossrefPubMedGoogle Scholar
  • 63 Fabbri VP, Caporalini C, Asioli S, Buccoliero A. Paediatric-type diffuse low-grade gliomas: a clinically and biologically distinct group of tumours with a favourable outcome. Pathologica 2022; 114 (06) 410-421
    CrossrefPubMedGoogle Scholar
  • 64 Kasper LH, Baker SJ. Invited review: emerging functions of histone H3 mutations in paediatric diffuse high-grade gliomas. Neuropathol Appl Neurobiol 2020; 46 (01) 73-85
    CrossrefPubMedGoogle Scholar
  • 65 Sturm D, Witt H, Hovestadt V. et al. Hotspot mutations in H3F3A and IDH1 define distinct epigenetic and biological subgroups of glioblastoma. Cancer Cell 2012; 22 (04) 425-437
    CrossrefPubMedGoogle Scholar
  • 66 Jakacki RI, Cohen KJ, Buxton A. et al. Phase 2 study of concurrent radiotherapy and temozolomide followed by temozolomide and lomustine in the treatment of children with high-grade glioma: a report of the Children's Oncology Group ACNS0423 study. Neuro-oncol 2016; 18 (10) 1442-1450
    CrossrefPubMedGoogle Scholar
  • 67 López-Pérez CA, Franco-Mojica X, Villanueva-Gaona R, Díaz-Alba A, Rodríguez-Florido MA, Navarro VG. Adult diffuse midline gliomas H3 K27-altered: review of a redefined entity. J Neurooncol 2022; 158 (03) 369-378
    CrossrefPubMedGoogle Scholar
  • 68 Schulte JD, Buerki RA, Lapointe S. et al. Clinical, radiologic, and genetic characteristics of histone H3 K27M-mutant diffuse midline gliomas in adults. Neurooncol Adv 2020; 2 (01) vdaa142
    CrossrefPubMedGoogle Scholar
  • 69 Picart T, Barritault M, Poncet D. et al. Characteristics of diffuse hemispheric gliomas, H3 G34-mutant in adults. Neurooncol Adv 2021; 3 (01) vdab061
    PubMedGoogle Scholar
  • 70 Crowell C, Mata-Mbemba D, Bennett J. et al. Systematic review of diffuse hemispheric glioma, H3 G34-mutant: outcomes and associated clinical factors. Neurooncol Adv 2022; 4 (01) vdac133
    PubMedGoogle Scholar
  • 71 Kurokawa R, Baba A, Kurokawa M. et al. Neuroimaging features of diffuse hemispheric glioma, H3 G34-mutant: a case series and systematic review. J Neuroimaging 2022; 32 (01) 17-27
    CrossrefPubMedGoogle Scholar
  • 72 Hong L, Shi ZF, Li KK. et al. Molecular landscape of pediatric type IDH wildtype, H3 wildtype hemispheric glioblastomas. Lab Invest 2022; 102 (07) 731-740
    CrossrefPubMedGoogle Scholar
  • 73 Korshunov A, Schrimpf D, Ryzhova M. et al. H3-/IDH-wild type pediatric glioblastoma is comprised of molecularly and prognostically distinct subtypes with associated oncogenic drivers. Acta Neuropathol 2017; 134 (03) 507-516
    CrossrefPubMedGoogle Scholar
  • 74 Mezzacappa FM, Thorell W. Neuronal Brain Tumors. Treasure Island, FL:: StatPearls Publishing Copyright © 2022, StatPearls Publishing LLC.; 2022
    Google Scholar
  • 75 Stone TJ, Keeley A, Virasami A. et al. Comprehensive molecular characterisation of epilepsy-associated glioneuronal tumours. Acta Neuropathol 2018; 135 (01) 115-129
    CrossrefPubMedGoogle Scholar
  • 76 Mallick S, Benson R, Melgandi W, Giridhar P, Rath GK. Impact of surgery, adjuvant treatment, and other prognostic factors in the management of anaplastic ganglioglioma. Childs Nerv Syst 2018; 34 (06) 1207-1213
    CrossrefPubMedGoogle Scholar
  • 77 Reinhardt A, Pfister K, Schrimpf D. et al. Anaplastic ganglioglioma-a diagnosis comprising several distinct tumour types. Neuropathol Appl Neurobiol 2022; 48 (07) e12847
    CrossrefPubMedGoogle Scholar
  • 78 Englot DJ, Han SJ, Berger MS, Barbaro NM, Chang EF. Extent of surgical resection predicts seizure freedom in low-grade temporal lobe brain tumors. Neurosurgery 2012; 70 (04) 921-928 , discussion 928
    CrossrefPubMedGoogle Scholar
  • 79 Haydon DH, Dahiya S, Smyth MD, Limbrick DD, Leonard JR. Greater extent of resection improves ganglioglioma recurrence-free survival in children: a volumetric analysis. Neurosurgery 2014; 75 (01) 37-42
    CrossrefPubMedGoogle Scholar
  • 80 Hu Y, Zhang H, Adilijiang A. et al. Seizure outcomes and prognostic factors in patients with gangliogliomas associated with epilepsy. Front Surg 2022; 9: 946201
    CrossrefPubMedGoogle Scholar
  • 81 Vasiljevic A, François P, Loundou A. et al. Prognostic factors in central neurocytomas: a multicenter study of 71 cases. Am J Surg Pathol 2012; 36 (02) 220-227
    CrossrefPubMedGoogle Scholar
  • 82 Chen YD, Li WB, Feng J, Qiu XG. Long-term outcomes of adjuvant radiotherapy after surgical resection of central neurocytoma. Radiat Oncol 2014; 9: 242
    CrossrefPubMedGoogle Scholar
  • 83 Johnson MO, Kirkpatrick JP, Patel MP. et al. The role of chemotherapy in the treatment of central neurocytoma. CNS Oncol 2019; 8 (03) CNS41
    CrossrefPubMedGoogle Scholar
  • 84 Samhouri L, Meheissen MAM, Ibrahimi AKH. et al. Impact of adjuvant radiotherapy in patients with central neurocytoma: a multicentric international analysis. Cancers (Basel) 2021; 13 (17) 4308
    CrossrefPubMedGoogle Scholar
  • 85 Beland B, Tsang RY, Sutherland G. Unprecedented response to combination BRAF and MEK inhibitors in adult anaplastic ganglioglioma. J Neurooncol 2018; 137 (03) 667-669
    CrossrefPubMedGoogle Scholar
  • 86 Berzero G, Bellu L, Baldini C. et al. Sustained tumor control with MAPK inhibition in BRAF V600-mutant adult glial and glioneuronal tumors. Neurology 2021; 97 (07) e673-e683
    CrossrefPubMedGoogle Scholar
  • 87 Marks AM, Bindra RS, DiLuna ML. et al. Response to the BRAF/MEK inhibitors dabrafenib/trametinib in an adolescent with a BRAF V600E mutated anaplastic ganglioglioma intolerant to vemurafenib. Pediatr Blood Cancer 2018; 65 (05) e26969
    CrossrefPubMedGoogle Scholar
  • 88 Rush S, Foreman N, Liu A. Brainstem ganglioglioma successfully treated with vemurafenib. J Clin Oncol 2013; 31 (10) e159-e160
    CrossrefPubMedGoogle Scholar
  • 89 Shih KC, Shastry M, Williams JT. et al. Successful treatment with dabrafenib (GSK2118436) in a patient with ganglioglioma. J Clin Oncol 2014; 32 (29) e98-e100
    CrossrefPubMedGoogle Scholar
  • 90 Gerstner ER, Pajtler KW. Ependymoma. Semin Neurol 2018; 38 (01) 104-111
    Article in Thieme ConnectPubMedGoogle Scholar
  • 91 Rudà R, Bruno F, Pellerino A, Soffietti R. Ependymoma: evaluation and management updates. Curr Oncol Rep 2022; 24 (08) 985-993
    CrossrefPubMedGoogle Scholar
  • 92 Ghasemi DR, Sill M, Okonechnikov K. et al. MYCN amplification drives an aggressive form of spinal ependymoma. Acta Neuropathol 2019; 138 (06) 1075-1089
    CrossrefPubMedGoogle Scholar
  • 93 Benesch M, Frappaz D, Massimino M. Spinal cord ependymomas in children and adolescents. Childs Nerv Syst 2012; 28 (12) 2017-2028
    CrossrefPubMedGoogle Scholar
  • 94 Bertero L, Ricci AA, Tampieri C, Cassoni P, Modena P. Ependymomas. Pathologica 2022; 114 (06) 436-446
    CrossrefPubMedGoogle Scholar
  • 95 Svoboda N, Bradac O, de Lacy P, Benes V. Intramedullary ependymoma: long-term outcome after surgery. Acta Neurochir (Wien) 2018; 160 (03) 439-447
    CrossrefPubMedGoogle Scholar
  • 96 Pajtler KW, Mack SC, Ramaswamy V. et al. The current consensus on the clinical management of intracranial ependymoma and its distinct molecular variants. Acta Neuropathol 2017; 133 (01) 5-12
    CrossrefPubMedGoogle Scholar
  • 97 Rudà R, Reifenberger G, Frappaz D. et al. EANO guidelines for the diagnosis and treatment of ependymal tumors. Neuro-oncol 2018; 20 (04) 445-456
    CrossrefPubMedGoogle Scholar
  • 98 Central Nervous System Cancers. National Comprehensive Cancer Network. 2022 [updated September 29, 2022 ]. Accessed October 27, 2023 at: https://www.nccn.org/professionals/physician_gls/pdf/cns.pdf
    PubMedGoogle Scholar
  • 99 Lötsch D, Kirchhofer D, Englinger B. et al. Targeting fibroblast growth factor receptors to combat aggressive ependymoma. Acta Neuropathol 2021; 142 (02) 339-360
    CrossrefPubMedGoogle Scholar
  • 100 Louis DN, Perry A, Reifenberger G. et al. The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol 2016; 131 (06) 803-820
    CrossrefPubMedGoogle Scholar
  • 101 Cotter JA, Hawkins C. Medulloblastoma: WHO 2021 and beyond. Pediatr Dev Pathol 2022; 25 (01) 23-33
    CrossrefPubMedGoogle Scholar
  • 102 Franceschi E, Seidel C, Sahm F, Pajtler KW, Hau P. How we treat medulloblastoma in adults. ESMO Open 2021; 6 (04) 100173
    CrossrefPubMedGoogle Scholar
  • 103 Frappaz D, Faure-Conter C, Bonneville Levard A, Barritault M, Meyronet D, Sunyach MP. Medulloblastomas in adolescents and adults - can the pediatric experience be extrapolated?. Neurochirurgie 2021; 67 (01) 76-82
    CrossrefPubMedGoogle Scholar
  • 104 Lazow MA, Palmer JD, Fouladi M, Salloum R. Medulloblastoma in the modern era: review of contemporary trials, molecular advances, and updates in management. Neurotherapeutics 2022; 19 (06) 1733-1751
    CrossrefPubMedGoogle Scholar
  • 105 Coltin H, Sundaresan L, Smith KS. et al. Subgroup and subtype-specific outcomes in adult medulloblastoma. Acta Neuropathol 2021; 142 (05) 859-871
    CrossrefPubMedGoogle Scholar
  • 106 Franceschi E, Giannini C, Furtner J. et al. Adult Medulloblastoma: updates on current management and future perspectives. Cancers (Basel) 2022; 14 (15) 3708
    CrossrefPubMedGoogle Scholar
  • 107 Goschzik T, Zur Muehlen A, Doerner E. et al. Medulloblastoma in adults: cytogenetic phenotypes identify prognostic subgroups. J Neuropathol Exp Neurol 2021; 80 (05) 419-430
    CrossrefPubMedGoogle Scholar
  • 108 Franceschi E, Hofer S, Brandes AA. et al. EANO-EURACAN clinical practice guideline for diagnosis, treatment, and follow-up of post-pubertal and adult patients with medulloblastoma. Lancet Oncol 2019; 20 (12) e715-e728
    CrossrefPubMedGoogle Scholar
  • 109 Fang FY, Rosenblum JS, Ho WS, Heiss JD. New developments in the pathogenesis, therapeutic targeting, and treatment of pediatric medulloblastoma. Cancers (Basel) 2022; 14 (09) 2285
    CrossrefPubMedGoogle Scholar
  • 110 Waszak SM, Northcott PA, Buchhalter I. et al. Spectrum and prevalence of genetic predisposition in medulloblastoma: a retrospective genetic study and prospective validation in a clinical trial cohort. Lancet Oncol 2018; 19 (06) 785-798
    CrossrefPubMedGoogle Scholar
  • 111 Warren KE, Vezina G, Poussaint TY. et al. Response assessment in medulloblastoma and leptomeningeal seeding tumors: recommendations from the Response Assessment in Pediatric Neuro-Oncology committee. Neuro-oncol 2018; 20 (01) 13-23
    CrossrefPubMedGoogle Scholar
  • 112 Thompson EM, Hielscher T, Bouffet E. et al. Prognostic value of medulloblastoma extent of resection after accounting for molecular subgroup: a retrospective integrated clinical and molecular analysis. Lancet Oncol 2016; 17 (04) 484-495
    CrossrefPubMedGoogle Scholar
  • 113 Zhang N, Ouyang T, Kang H, Long W, Thomas B, Zhu S. Adult medulloblastoma: clinical characters, prognostic factors, outcomes and patterns of relapse. J Neurooncol 2015; 124 (02) 255-264
    CrossrefPubMedGoogle Scholar
  • 114 Penas-Prado M, Theeler BJ, Cordeiro B. et al. Proceedings of the Comprehensive Oncology Network Evaluating Rare CNS Tumors (NCI-CONNECT) Adult Medulloblastoma Workshop. Neurooncol Adv 2020; 2 (01) vdaa097
    PubMedGoogle Scholar
  • 115 Cooney T, Lindsay H, Leary S, Wechsler-Reya R. Current studies and future directions for medulloblastoma: a review from the pacific pediatric neuro-oncology consortium (PNOC) disease working group. Neoplasia 2023; 35: 100861
    CrossrefPubMedGoogle Scholar
  • 116 Michalski JM, Janss AJ, Vezina LG. et al. Children's oncology group phase III trial of reduced-dose and reduced-volume radiotherapy with chemotherapy for newly diagnosed average-risk medulloblastoma. J Clin Oncol 2021; 39 (24) 2685-2697
    CrossrefPubMedGoogle Scholar
  • 117 Packer RJ, Gajjar A, Vezina G. et al. Phase III study of craniospinal radiation therapy followed by adjuvant chemotherapy for newly diagnosed average-risk medulloblastoma. J Clin Oncol 2006; 24 (25) 4202-4208
    CrossrefPubMedGoogle Scholar
  • 118 Hwang EI, Kool M, Burger PC. et al. Extensive molecular and clinical heterogeneity in patients with histologically diagnosed CNS-PNET treated as a single entity: a report from the children's oncology group randomized ACNS0332 trial. J Clin Oncol 2018; 36 (34) JCO2017764720
    CrossrefPubMedGoogle Scholar
  • 119 Tarbell NJ, Friedman H, Polkinghorn WR. et al. High-risk medulloblastoma: a pediatric oncology group randomized trial of chemotherapy before or after radiation therapy (POG 9031). J Clin Oncol 2013; 31 (23) 2936-2941
    CrossrefPubMedGoogle Scholar
  • 120 Esbenshade AJ, Kocak M, Hershon L. et al. A phase II feasibility study of oral etoposide given concurrently with radiotherapy followed by dose intensive adjuvant chemotherapy for children with newly diagnosed high-risk medulloblastoma (protocol POG 9631): a report from the Children's Oncology Group. Pediatr Blood Cancer 2017; 64 (06) 10 .1002/pbc.26373
    CrossrefPubMedGoogle Scholar
  • 121 Brandes AA, Franceschi E, Tosoni A, Blatt V, Ermani M. Long-term results of a prospective study on the treatment of medulloblastoma in adults. Cancer 2007; 110 (09) 2035-2041
    CrossrefPubMedGoogle Scholar
  • 122 Kocakaya S, Beier CP, Beier D. Chemotherapy increases long-term survival in patients with adult medulloblastoma–a literature-based meta-analysis. Neuro-oncol 2016; 18 (03) 408-416
    CrossrefPubMedGoogle Scholar
  • 123 Packer RJ, Sutton LN, Elterman R. et al. Outcome for children with medulloblastoma treated with radiation and cisplatin, CCNU, and vincristine chemotherapy. J Neurosurg 1994; 81 (05) 690-698
    CrossrefPubMedGoogle Scholar
  • 124 Yan H, Zabih V, Bartels U, Das S, Nathan P, Gupta S. Prognostic factors related to overall survival in adolescent and young adults with medulloblastoma: a systematic review. Neurooncol Adv 2022; 4 (01) vdac016
    PubMedGoogle Scholar
  • 125 Robinson GW, Orr BA, Wu G. et al. Vismodegib exerts targeted efficacy against recurrent sonic hedgehog-subgroup medulloblastoma: results from phase II pediatric brain tumor consortium studies PBTC-025B and PBTC-032. J Clin Oncol 2015; 33 (24) 2646-2654
    CrossrefPubMedGoogle Scholar
  • 126 Kieran MW, Chisholm J, Casanova M. et al. Phase I study of oral sonidegib (LDE225) in pediatric brain and solid tumors and a phase II study in children and adults with relapsed medulloblastoma. Neuro-oncol 2017; 19 (11) 1542-1552
    CrossrefPubMedGoogle Scholar
  • 127 Bromberg JE, Baumert BG, de Vos F. et al. Primary intracranial germ-cell tumors in adults: a practical review. J Neurooncol 2013; 113 (02) 175-183
    CrossrefPubMedGoogle Scholar
  • 128 Frappaz D, Dhall G, Murray MJ. et al. EANO, SNO and Euracan consensus review on the current management and future development of intracranial germ cell tumors in adolescents and young adults. Neuro-oncol 2022; 24 (04) 516-527
    CrossrefPubMedGoogle Scholar
  • 129 Gittleman H, Cioffi G, Vecchione-Koval T. et al. Descriptive epidemiology of germ cell tumors of the central nervous system diagnosed in the United States from 2006 to 2015. J Neurooncol 2019; 143 (02) 251-260
    CrossrefPubMedGoogle Scholar
  • 130 Murray MJ, Bartels U, Nishikawa R, Fangusaro J, Matsutani M, Nicholson JC. Consensus on the management of intracranial germ-cell tumours. Lancet Oncol 2015; 16 (09) e470-e477
    CrossrefPubMedGoogle Scholar
  • 131 Jennings MT, Gelman R, Hochberg F. Intracranial germ-cell tumors: natural history and pathogenesis. J Neurosurg 1985; 63 (02) 155-167
    CrossrefPubMedGoogle Scholar
  • 132 Yeo KK, Nagabushan S, Dhall G, Abdelbaki MS. Primary central nervous system germ cell tumors in children and young adults: a review of controversies in diagnostic and treatment approach. Neoplasia 2023; 36: 100860
    CrossrefPubMedGoogle Scholar
  • 133 Byun HK, Yoon HI, Cho J. et al. Optimization of intracranial germinoma treatment: radiotherapy alone with reduced volume and dose. Int J Radiat Oncol Biol Phys 2020; 108 (03) 657-666
    CrossrefPubMedGoogle Scholar
  • 134 Bartels U, Onar-Thomas A, Patel SK. et al. Phase II trial of response-based radiation therapy for patients with localized germinoma: a Children's Oncology Group study. Neuro-oncol 2022; 24 (06) 974-983
    CrossrefPubMedGoogle Scholar
  • 135 Calaminus G, Kortmann R, Worch J. et al. SIOP CNS GCT 96: final report of outcome of a prospective, multinational nonrandomized trial for children and adults with intracranial germinoma, comparing craniospinal irradiation alone with chemotherapy followed by focal primary site irradiation for patients with localized disease. Neuro-oncol 2013; 15 (06) 788-796
    CrossrefPubMedGoogle Scholar
  • 136 Fonseca A, Faure-Conter C, Murray MJ. et al. Pattern of treatment failures in patients with central nervous system non-germinomatous germ cell tumors (CNS-NGGCT): a pooled analysis of clinical trials. Neuro-oncol 2022; 24 (11) 1950-1961
    CrossrefPubMedGoogle Scholar
  • 137 Yeo KK, Burgers DE, Brodigan K. et al. Adolescent and young adult neuro-oncology: a comprehensive review. Neurooncol Pract 2021; 8 (03) 236-246
    PubMedGoogle Scholar