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Table 1 Syndromes causing predisposition to Wilms' Tumor

From: In silico regulatory analysis for exploring human disease progression

Syndrome

Occurrence of Wilms tumor

Chromosomal abnormality

Ref.

WAGR

98% by age 6

Deletion at 11p13

OMIM: #194072

Beckwith- Wiedemann

96% by age 8

Duplication of paternal 11p15. May result in increased gene expression(IGF2) or inactivation(p57).

OMIM: #130650

Denys-Drash

96% by age 5

Missense mutation in WT1 (11p13 locus) causing dominant negative phenotype.

OMIM: #194080

  1. This table highlights the syndromes causing predisposition to Wilms' Tumor development, and the genetic changes associated with the syndrome. The reference number for the syndrome in the Online Mendelian Inheritance in Man (OMIM) database [231] is given in the Ref. column. These include WAGR [139] Denys-Drash [232], and Beckwith-Wiedemann [140] syndromes.