Botyroid Wilms Tumor

In some cases, Wilms tumor may protrude into the calyces forming polypoid masses resembling botyroid rhabdomyosarcoma. In addition to WAGR Syndrome (see previous slide), patients with Denys-Drash Syndrome have a high risk of developing Wilms tumor. This syndrome is characterized by male pseudohermaphroditism and diffuse mesangial sclerosis leading to early renal failure. Patients with Denys-Drash syndrome harbor a germline mutation in the zinc-finger region of the WT1 protein which affects its DNA-binding properties. These patients also harbor increased risk of developing gonadoblastomas. Image courtesy of Dr. Jean-Christophe Fournet, Paris, France; humpath.com; Used with permission