Significance of TP53 Mutation in Wilms Tumors with Diffuse Anaplasia: A Report from the Children's Oncology Group.

ABSTRACT:

Purpose

To investigate the role and significance of TP53 mutation in diffusely anaplastic Wilms tumors (DAWTs).

Experimental design

All DAWTs registered on National Wilms Tumor Study-5 (n = 118) with available samples were analyzed for TP53 mutations and copy loss. Integrative genomic analysis was performed on 39 selected DAWTs.

Results

Following analysis of a single random sample, 57 DAWTs (48%) demonstrated TP53 mutations, 13 (11%) copy loss without mutation, and 48 (41%) lacked both [defined as TP53-wild-type (wt)]. Patients with stage III/IV TP53-wt DAWTs (but not those with stage I/II disease) had significantly lower relapse and death rates than those with TP53 abnormalities. In-depth analysis of a subset of 39 DAWTs showed seven (18%) to be TP53-wt: These demons

SUBMITTER: Ooms AH 

PROVIDER: S-EPMC5290091 | biostudies-literature | 2016 Nov

REPOSITORIES: biostudies-literature