Which condition is associated with the WT1 gene mutation and commonly leads to Wilms tumor?

The condition associated with the WT1 gene mutation that commonly leads to Wilms tumor is Denys-Drash Syndrome. This syndrome is characterized by a triad of findings: nephropathy (which can lead to end-stage renal disease), genital anomalies, and a predisposition to Wilms tumor, which is a type of kidney cancer typically seen in children. The WT1 gene plays a crucial role in kidney and gonadal development, and mutations can disrupt normal development, increasing the risk of these associated conditions.

Denys-Drash Syndrome exemplifies the direct link between the WT1 gene and renal tumors, notably Wilms tumor, due to the gene's critical functions in organogenesis. The connection between the mutation and the tumor is well-established, making this condition a prime example of genetic factors leading to specific pediatric tumors.

In contrast, the other conditions listed do not have the same strong association with Wilms tumor. Frasier Syndrome, while also linked to the WT1 gene, primarily involves issues related to gonadal development and does not present the same high risk for Wilms tumor. Congenital Adrenal Hyperplasia is primarily a disorder of adrenal hormone synthesis and is not connected to WT1 mutations or kidney tumors. CHARGE Syndrome encompasses