Overview

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

MYCN +

Disease Details

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

References

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.