Reconciling Oncologic Outcomes With Renal Preservation in Denys-Drash Syndrome and Recurrent Wilms Tumor: A Case-Based Discussion.
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Denys Drash syndrome (DDS) results from a mutation in the WT1 tumor suppressor gene manifesting early in childhood. The classic presentation is characterized by early end-stage renal disease (ESRD), differences of sexual differentiation (DSD), and high risk of Wilms tumor (WT). Management varies based on individual patient presentations. We present a rare case of an adult with DDS who presented with functional, native renal tissue and a new renal mass, who was previously treated for WT in childhood. We discuss this unique clinical presentation and review the complex management of patients with DDS primarily as it relates to WT.