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Pediatr Blood Cancer. 2013 Sep;60(9):1408-10. doi: 10.1002/pbc.24511. Epub 2013 Mar 19.

Implications of tumor location on subtypes of medulloblastoma.

Author information

1
Department of Pediatrics, Division of Hematology-Oncology, Texas Children's Cancer Center, Baylor College of Medicine, Houston, Texas, USA.

Abstract

BACKGROUND:

Medulloblastoma (MB) comprises of four molecular subtypes, Sonic hedgehog (SHH), Wingless (WNT), Groups 3 and 4. WNT-subtype MBs were found to arise from midline of the brainstem occupying the fourth ventricle while SHH-subtype occupied the cerebellar hemisphere in a small subset of patients.

PROCEDURE:

We tested this hypothesis in a large cohort of pediatric MBs comprising of all four molecular subtypes.

RESULTS:

We validated in the first comprehensive analysis of tumor location of 60 human MBs representative of the four molecular subtypes, that hemispheric tumors are significantly associated with SHH-subtype MBs while midline tumors with WNT-subtype, Group 3 and 4 MBs (P < 0.001). Nearly half of SHH-subtype MBs were midline.

CONCLUSIONS:

Tumor location should not be generalized to MB subtypes. SHH-subtype MBs are not exclusively hemispheric and hemispheric MBs are not always SHH-activated. It is imperative to identify subtypes in conjunction with tumor location when exploring currently available targeted therapy.

KEYWORDS:

medulloblastoma; molecular subtypes; pediatric; tumor location

PMID:
23512859
DOI:
10.1002/pbc.24511
[Indexed for MEDLINE]

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