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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.