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Review
. 2014 Jul 8:6:56.
doi: 10.12703/P6-56. eCollection 2014.

Advances in managing medulloblastoma and intracranial primitive neuro-ectodermal tumors

Jenny Adamski  1 , Vijay Ramaswamy  2 , Annie Huang  3 , Eric Bouffet  1
Affiliations
Review

Advances in managing medulloblastoma and intracranial primitive neuro-ectodermal tumors

Jenny Adamski et al. F1000Prime Rep. .

Abstract

Medulloblastoma and central nervous system (CNS)-primitive neuro-ectodermal tumors (PNETs) are a diverse group of entities which encompasses different pathological and clinical pictures. Initially divided based on histology and location, molecular insight is leading to new definitions and a change in the borders delineating these diseases, such that they become more divergent. Current treatment approaches consist of surgical resection, radiotherapy and intensive chemotherapy, dependent on age. Stratification is one risk factor shown to be prognostic and is divided into high- and average-risks. Outcomes with modern treatment regimens are good, particularly in average-risk medulloblastoma patients, but the cost of cure is high, with high rates of neurocognitive, endocrine and social dysfunction. The changing biological landscape, however, may allow for clearer prediction of tumor behavior, to better identify "good" and "bad" players within these groups. Discovery of subgroups with changes in dependent molecular pathways will also lead to the development of new specific targeted therapies. Presenting exciting opportunities, these advances may transform the treatment for some patients, revolutionizing therapy in the future. Several challenges, however, are yet to be faced and caution is needed not to abandon previously defined prognostic factors on the strength of thus far retrospective evidence. We are witnessing a new era of trials with biological stratification involving multiple subgroups and treatment arms, based on specific tumor-related targets. This review discusses the changing face of medulloblastoma and CNS-PNETs and how we move molecular advances into clinical trials that benefit patients.

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Figures

Figure 1.
Figure 1.. MRI images of medulloblastoma
T1 weighted magnetic resonance imaging (MRI) images are shown following contrast administration. A) shows an axial image of a 6 year old child with a classical medulloblastoma. B) shows an axial image of an 8 month old child with medulloblastoma with extensive nodularity (MBEN).
Figure 2.
Figure 2.. MRI images of CNS-PNET
T1 weighted magnetic resonance imaging (MRI) images are shown following contrast administration. A) shows an axial image of a 4-year old child with an ETANTR (embryonal tumor with abundant neuropil and true rosettes). B) shows an axial image of an 8-month old child with medulloepithelioma. C) shows an axial image of a 6-year old child with a supratentorial PNET. D) shows a sagittal image of a 9-month old child with pineoblastoma.
Figure 3.
Figure 3.. Medulloblastoma subgrouping
The current consensus on medulloblastoma subgrouping reproduced from Taylor et al. 2012 [8]. SHH, sonic hedgehog.
Figure 4.
Figure 4.. The future treatment of medulloblastoma
Proposed future treatments based on molecular subgrouping adapted from Ramaswarmy et al. 2011 [68]. CSI, craniospinal radiation; CNS-PNET, central nervous system primitive neuro-ectodermal tumor; ETANTR, embryonal tumor with abundant neuropil and true rosettes; SHH, sonic hedgehog.
See this image and copyright information in PMC

References

    1. Rorke LB. The cerebellar medulloblastoma and its relationship to primitive neuroectodermal tumors. J Neuropathol Exp Neurol. 1983;42:1–15. doi: 10.1097/00005072-198301000-00001. - DOI - PubMed
    1. Louis D, Ohgaki H, Wiestler O, Cavenee W. WHO classification of tumours of the central nervous system. Lyon: IARC Press; 2007. - PMC - PubMed
    1. Pomeroy SL, Tamayo P, Gaasenbeek M, Sturla LM, Angelo M, McLaughlin ME, Kim JY, Goumnerova LC, Black PM, Lau C, Allen JC, Zagzag D, Olson JM, Curran T, Wetmore C, Biegel JA, Poggio T, Mukherjee S, Rifkin R, Califano A, Stolovitzky G, Louis DN, Mesirov JP, Lander ES, Golub TR. Prediction of central nervous system embryonal tumour outcome based on gene expression. Nature. 2002;415:436–42. doi: 10.1038/415436a. - DOI - PubMed

    2. http://f1000.com/prime/1003625

    1. Cho YJ, Tsherniak A, Tamayo P, Santagata S, Ligon A, Greulich H, Berhoukim R, Amani V, Goumnerova L, Eberhart CG, Lau CC, Olson JM, Gilbertson RJ, Gajjar A, Delattre O, Kool M, Ligon K, Meyerson M, Mesirov JP, Pomeroy SL. Integrative genomic analysis of medulloblastoma identifies a molecular subgroup that drives poor clinical outcome. J Clin Oncol. 2011;29:1424–30. doi: 10.1200/JCO.2010.28.5148. - DOI - PMC - PubMed
    1. Kool M, Koster J, Bunt J, Hasselt NE, Lakeman A, van Sluis P, Troost D, Meeteren NS, Caron HN, Cloos J, Mrsić A, Ylstra B, Grajkowska W, Hartmann W, Pietsch T, Ellison D, Clifford SC, Versteeg R. Integrated genomics identifies five medulloblastoma subtypes with distinct genetic profiles, pathway signatures and clinicopathological features. PLoS One. 2008;3:e3088. doi: 10.1371/journal.pone.0003088. - DOI - PMC - PubMed