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Review
. 2016 Nov 15;56(11):687-697.
doi: 10.2176/nmc.ra.2016-0016. Epub 2016 May 26.

Molecular Classification of Medulloblastoma

Noriyuki Kijima  1 , Yonehiro Kanemura
Affiliations
Review

Molecular Classification of Medulloblastoma

Noriyuki Kijima et al. Neurol Med Chir (Tokyo). .

Abstract

Medulloblastoma (MB) is one of the most frequent malignant brain tumors in children. The current standard treatment regimen consists of surgical resection, craniospinal irradiation, and adjuvant chemotherapy. Although these treatments have the potential to increase the survival of 70-80% of patients with MB, they are also associated with serious treatment-induced morbidity. The current risk stratification of MB is based on clinical factors, including age at presentation, metastatic status, and the presence of residual tumor following resection. In addition, recent genomic studies indicate that MB consists of at least four distinct molecular subgroups: WNT, sonic hedgehog (SHH), Group 3, and Group 4. WNT and SHH MBs are characterized by aberrations in the WNT and SHH signaling pathways, respectively. WNT MB has the best prognosis compared to the other MBs, while SHH MB has an intermediate prognosis. The underlying signaling pathways associated with Group 3 and 4 MBs have not been identified. Group 3 MB is frequently associated with metastasis, resulting in a poor prognosis, while Group 4 is sometimes associated with metastasis and has an intermediate prognosis. Group 4 is the most frequent MB and represents 35% of all MBs. These findings suggest that MB is a heterogeneous disease, and that MB subgroups have distinct molecular, demographic, and clinical characteristics. The molecular classification of MBs is redefining the risk stratification of patients with MB, and has the potential to identify new therapeutic strategies for the treatment of MB.

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Conflict of interest statement

The authors have no conflict of interest.

Figures

Fig. 1
Fig. 1
Newly proposed risk stratification systems for MB patients. (A) Risk stratification proposed by Pietsch et al. (B) Risk stratification proposed by Shih et al. (C) Risk stratification proposed by Gottardo et al. LCA: large cell anaplastic, M: metastatic status, GTR: gross total resection, NTR: near total resection.
Fig. 2
Fig. 2
MB localization of the various MB subgroups.
See this image and copyright information in PMC

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