# Subgroup-specific prognostic implications of TP53 mutation in medulloblastoma

Source: https://onco.cc/key-papers/paper-zhukova-tp53-medulloblastoma-jco-2013/  
OnCo record `paper-zhukova-tp53-medulloblastoma-jco-2013` (Key paper). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

TP53 mutations mark a very poor prognosis in SHH-subgroup medulloblastoma, with five-year survival around 40 percent, but have no effect in WNT tumours, so the mutation must be interpreted alongside the subgroup.

## Summary

Analysis of 553 medulloblastomas for TP53 mutation by molecular subgroup in a discovery and validation cohort, finding TP53 mutations in 21 percent of SHH and 16 percent of WNT tumours but rarely in groups 3 and 4.

In SHH tumours, TP53 mutation was associated with five-year overall survival of 41 percent against 81 percent for wild-type, often with germline mutations (Li-Fraumeni syndrome) and chromothripsis, whereas WNT tumours with TP53 mutations retained excellent survival.

## Fields

- Kind: Key paper
- Last checked: 2026-09-17
- Journal: Journal of Clinical Oncology
- Year: 2013
- DOI: 10.1200/JCO.2012.48.5052
- Authors: Zhukova N, Ramaswamy V, Remke M, et al.
- Findings: SHH TP53-mutant: five-year overall survival 41 percent vs 81 percent for wild-type.; WNT TP53-mutant: survival unaffected (90 percent).
- What it means: SHH-activated, TP53-mutant medulloblastoma is a separate WHO entity treated as very high risk, and its diagnosis prompts germline testing of the child and family.
- Caveats: Retrospective; treatment varied across cohorts.

## Sources

- J Clin Oncol 2013: https://doi.org/10.1200/JCO.2012.48.5052
- PubMed: https://pubmed.ncbi.nlm.nih.gov/23835706/

## Connected records

- cancers: [SHH-activated medulloblastoma](https://onco.cc/cancers/medulloblastoma-shh/)
- journals: [Journal of Clinical Oncology](https://onco.cc/journals/jco/)

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