NCI Definition: An embryonal pediatric tumor of the kidney which may also be seen rarely in adults. The peak incidence of Wilms tumor is between the second and fifth year of life. Microscopically, it is composed of a mixture of cellular elements (blastemal, stromal, and epithelial). The most common sites of metastasis include the regional lymph nodes, lungs, and liver. [1]

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

Most Commonly Altered Genes in Kidney 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 kidney Wilms tumor [2].

Top Alterations in Kidney Wilms Tumor

Significant Genes in Kidney Wilms Tumor


Disease Details

NEPHROBLASTOMA, MALIGNANT, Renal Wilms' Tumor, Wilms' tumor, Wilms' Tumor of the Kidney, Wilms Tumor of the Kidney, Nephroblastoma, Renal Wilms Tumor, Embryonal Nephroma
Wilms Tumor
Mixed Cell Type Kidney Wilms Tumor, Nonanaplastic Kidney Wilms Tumor, Metachronous Wilms Tumor, Hereditary Wilms Tumor, Blastema Predominant Kidney Wilms Tumor, Epithelial Predominant Kidney Wilms Tumor, and Stromal Predominant Kidney Wilms Tumor
OncoTree Name
Wilms' Tumor
OncoTree Code


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.