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Comment
. 2020 Jan 11;22(1):7-9.
doi: 10.1093/neuonc/noz213.

Less treatment for Wing less medulloblastoma: germline data re-emphasize this

Anirban Das  1 , Vijay Ramaswamy  1   2
Affiliations
Comment

Less treatment for Wing less medulloblastoma: germline data re-emphasize this

Anirban Das et al. Neuro Oncol. .
No abstract available

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Figures

Fig. 1
Fig. 1
A diagnosis of WNT MB, confirmed by a combination of strong nuclear immunopositivity for β-catenin and/or presence of monosomy 6 by fluorescence in situ hybridization, should be followed by sequencing of the tumor for CTNNB1 mutation. Absence of a somatic CTNNB1 mutation should prompt germline testing for APC mutation. Presence of APC mutation should lead to enrollment in WNT MB specific trials, surveillance for second neoplasms, and genetic counseling. Both germline and somatic WNT MB share the same excellent prognosis following current multimodal therapy, albeit with neurocognitive sequelae. However, the overall survival continues to diminish beyond 10 years from the diagnosis due to higher risk of second malignancies, both related to the APC tumor predisposition syndrome, as well as due to therapy-related neoplasms.
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