Wilms Tumor After Orthotopic Liver Transplant in a Patient With Alagille Syndrome

We present a case of Wilms Tumor in a patient with Alagille syndrome 10 months after liver transplant. We explore a suggested genetic connection between these 2 diseases. In children with Wilms Tumor, we propose a pathoembryologic explanation for not just the tumor, but also for the cause of associated benign ureteral and renal parenchymal aberrancies that are commonly seen in the Alagille population. We also discuss the diagnostic and therapeutic challenges that can arise in a liver transplant patient with Alagille syndrome who subsequently develops a renal mass.