NCI Definition: An embryonal neoplasm characterized by the presence of epithelial, mesenchymal, and blastema components. The vast majority of cases arise from the kidney. A small number of cases with morphologic features resembling Wilms tumor of the kidney have been reported arising from the ovary and the cervix. [1]

Wilms tumors most frequently harbor alterations in TP53, MYCN, WT1, AMER1, and CTNNB1 [2].

Most Commonly Altered Genes in Wilms Tumor

TP53 Mutation, TP53 c.217-c.1178 Missense, TP53 Missense, CTNNB1 Mutation, and TP53 Exon 8 Mutation are the most common alterations in Wilms tumor [2].

Top Alterations in Wilms Tumor

Significant Genes in Wilms Tumor





Disease Details

Wilms tumor (Nephroblastoma), Wilms' Tumor
Malignant Mixed Neoplasm
Kidney Wilms Tumor, Ovarian Wilms Tumor, and Cervical Wilms Tumor


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.