Adult medulloblastoma comprises three major molecular variants. Remke M, Hielscher T, Northcott PA, Witt H, Ryzhova M, et al. Wnt/Wingless pathway activation and chromosome 6 loss characterize a distinct molecular subgroup of medulloblastomas associated with a favorable prognosis. 2017 49:780–8.Ĭlifford SC, Lusher ME, Lindsey JC, Langdon JA, Gilbertson RJ, Straughton D, et al. Spatial heterogeneity in medulloblastoma. Morrissy AS, Cavalli FMG, Remke M, Ramaswamy V, Shih DJH, Holgado BL, et al. Molecular subgroups of medulloblastoma: the current consensus. Taylor MD, Northcott PA, Korshunov A, Remke M, Cho YJ, Clifford SC, et al. Intertumoral heterogeneity within medulloblastoma subgroups. 2002 415:436–42.Ĭavalli FGM, Remke M, Rampasek L, Peacock J, Shih DJH, et al. Prediction of central nervous system embryonal tumour outcome based on gene expression. Pomeroy SL, Tamayo P, Gaasenbeek M, Sturla LM, Angelo M, McLaughlin ME, et al. It concluded that genetic counselling and testing should be a standard of care for WNT and SHH MB patients. 2018 19:785–98 This international, multicenter study found that the prevalence of genetic predisposition syndromes in MB patients was 6%, with SHH MB having the highest number of cases. Spectrum and prevalence of genetic predisposition in medulloblastoma: a retrospective genetic study and prospective validation in a clinical trial cohort. Non-significant trends in the data suggested disparities by race. Overall incidence rate remained stable with slight male predominance, and the incidence was highest in the youngest patients. 2017 135:433–41 The study examined population-level trends in MB incidence and survival using data from 2001 to 2013. Incidence and survival trends for medulloblastomas in the United States from 2001 to 2013. Khanna V, Achey RL, Ostrom QT, Block-Beach H, Kruchko C, Barnholtz-Sloan JS, et al. An integrative view on sex differences in brain tumors. CBTRUS statistical report: primary brain and other central nervous system tumors diagnosed in the United States in 2011-2015. Ostrom QT, Gittleman H, Truitt G, Boscia A, Kruchko C, Barnholtz-Sloan JS. Alex’s lemonade stand foundation infant and childhood primary brain and central nervous system tumors diagnosed in the United States in 2007–2011. Ostrom QT, de Blank PM, Kruchko C, Petersen CM, Liao P, Finlay JL, et al. The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, et al. Adjuvant chemotherapy for medulloblastoma: the first multicenter control trial of the International Society of Pediatric Oncology (SIOP I). Tait DM, Thornton-Jones H, Bloom HJ, Lemerle J, Morris-Jones P. Cerebellar medulloblastoma: treatment by irradiation of the whole central nervous system. Papers of particular interest, published recently, have been highlighted as: Recent innovations in medulloblastoma research allow us to better understand pathogenesis and molecular characteristics resulting in advanced risk stratification models, new therapeutic approaches, and overall improved survival and quality of life. Management of recurrent/refractory medulloblastoma remains a challenge with immunotherapy and small-molecule inhibitors forming the backbone of novel strategies. The mainstay of newly diagnosed medulloblastoma treatment is surgery, radiation therapy, and chemotherapy, except for children under 6 years of age, where high-dose chemotherapy with autologous stem cell rescue is used to avoid or delay radiotherapy, preventing neurocognitive sequelae. Risk stratification before and after the discovery of molecular subgroups aims at minimizing toxicity by reducing radiation and chemotherapy doses in low-risk patients while maintaining favorable overall survival (OS). Medulloblastoma comprises four molecularly distinct subgroups: wingless activated (WNT), sonic hedgehog activated (SHH), group 3, and group 4. This paper provides guidance for the clinician caring for pediatric and adult patients with medulloblastoma in the modern era. Molecular subtyping in medulloblastoma (MB) has diagnostic and prognostic values which impact therapy.
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