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Review
. 2018;16(7):1045-1058.
doi: 10.2174/1570159X15666171129111324.

Childhood Medulloblastoma: Current Therapies, Emerging Molecular Landscape and Newer Therapeutic Insights

Soumen Khatua  1 , Anne Song  2 , Divyaswathi Citla Sridhar  3 , Stephen C Mack  2
Affiliations
Review

Childhood Medulloblastoma: Current Therapies, Emerging Molecular Landscape and Newer Therapeutic Insights

Soumen Khatua et al. Curr Neuropharmacol. 2018.

Abstract

Background: Medulloblastoma is the most common malignant brain tumor in children, currently treated uniformly based on histopathology and clinico-radiological risk stratification leading to unpredictable relapses and therapeutic failures. Identification of molecular subgroups have thrown light on the reasons for these and now reveals clues to profile molecularly based personalized therapy against these tumors.

Methods: Research and online contents were evaluated for pediatric medulloblastoma which included latest information on the molecular subgroups and their clinical relevance and update on efforts to translate them into clinics.

Results: Scientific endeavors over the last decade have clearly identified four molecular variants (WNT, SHH, Group 3, and Group 4) and their demographic, genomic, and epigenetic profile. Latest revelations include significant heterogeneity within these subgroups and 12 different subtypes of MB are now identified with disparate outcomes and biology. These findings have important implications for stratification and profiling future clinical trials against these formidable tumors.

Conclusion: With the continued outpouring of genomic/epigenomic data of these molecular subgroups and evolution of further subtypes in each subgroup, the challenge lies in comprehensive evaluation of these informations. Current and future endeavors are now needed to profile personalized therapy for each child based on the molecular risk stratification of medulloblastoma, with a hope to improve survival outcome and reduce relapses.

Keywords: Childhood medulloblastoma; epigenetic machinery; molecular subtypes; newer treatment strategies; targeted therapy; therapeutic resistance..

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Figures

Fig. (1)
Fig. (1)
Treatment strategies based on molecular subtypes and risk factors in medulloblastoma.
See this image and copyright information in PMC

References

    1. Louis D.N., Perry A., Reifenberger G., von Deimling A., Figarella-Branger D., Cavenee W.K., Ohgaki H., Wiestler O.D., Kleihues P., Ellison D.W. The 2016 world health organization classification of tumors of the central nervous system: A summary. Acta Neuropathol. 2016;131(6):803–820. [http://dx.doi.org/ 10.1007/s00401-016-1545-1]. [PMID: 27157931]. - PubMed
    1. Eberhart C.G., Kepner J.L., Goldthwaite P.T., Kun L.E., Duffner P.K., Friedman H.S., Strother D.R., Burger P.C. Histopathologic grading of medulloblastomas: a Pediatric Oncology Group study. Cancer. 2002;94(2):552–560. [http://dx.doi.org/10.1002/ cncr.10189]. [PMID: 11900240]. - PubMed
    1. Perry A. Medulloblastomas with favorable versus unfavorable histology: how many small blue cell tumor types are there in the brain? Adv. Anat. Pathol. 2002;9(6):345–350. [http://dx.doi.org/ 10.1097/00125480-200211000-00003]. [PMID: 12409643]. - PubMed
    1. Gajjar A., Chintagumpala M., Ashley D., Kellie S., Kun L.E., Merchant T.E., Woo S., Wheeler G., Ahern V., Krasin M.J., Fouladi M., Broniscer A., Krance R., Hale G.A., Stewart C.F., Dauser R., Sanford R.A., Fuller C., Lau C., Boyett J.M., Wallace D., Gilbertson R.J. Risk-adapted craniospinal radiotherapy followed by high-dose chemotherapy and stem-cell rescue in children with newly diagnosed medulloblastoma (St Jude Medulloblastoma-96): long-term results from a prospective, multicentre trial. Lancet Oncol. 2006;7(10):813–820. [http://dx.doi.org/ 10.1016/S1470-2045(06)70867-1]. [PMID: 17012043]. - PubMed
    1. von Bueren A.O., von Hoff K., Pietsch T., Gerber N.U., Warmuth-Metz M., Deinlein F., Zwiener I., Faldum A., Fleischhack G., Benesch M., Krauss J., Kuehl J., Kortmann R.D., Rutkowski S. Treatment of young children with localized medulloblastoma by chemotherapy alone: results of the prospective, multicenter trial HIT 2000 confirming the prognostic impact of histology. Neuro-oncol. 2011;13(6):669–679. [http://dx.doi.org/10.1093/neuonc/ nor025]. [PMID: 21636711]. - PMC - PubMed