Management of Wilms' tumor with intracardiac extension. 94805 Villejuif, France. Since its initial description by Max Wilms over a century ago, nephroblastoma has benefited from considerable improvements both in terms of basic knowledge about it and management of it. The contribution of large international studies will be emphasized here. The Denys-Drash syndrome is defined by the occurrence of combinations of pseudohermaphroditism, nephrotic syndrome with diffuse mesangial sclerosis, Wilms' tumor, and constitutional mutations in the WT1 suppressor gene.
Most patients develop end-stage renal failure.
Each had nephrotic syndrome, diffuse mesangial sclerosis, and WT1 point mutations. Both had grade III hypospadias and undescended testes. Wilms tumor and beyond. A variety of pediatric renal masses may be differentiated from Wilms tumor on the basis of their clinical and imaging features. Nephroblastomatosis occurs most often in neonates and is characterized by multiple bilateral subcapsular masses, often associated with Wilms tumors.