Snapshot A 15-month-old male is brought in to his pediatrician for routine exam. On exam, his pediatrician notices that he has no irises, consistent with aniridia. He is also found with a palpable, non-tender mass on the left side of his abdomen. On further questioning, his mother reveals that her cousin had a similar abdominal mass at a young age. The patient and his mom are referred to pediatric surgeons for further management. An abdominal CT scan is obtained. Introduction Embryonal malignancy of the kidney login to view 3 more bullets Epidemiology Most common renal tumor of childhood Seen in otherwise healthy children < 4 years old ETIOLOGY Pathogenesis login to view 1 more bullet Risk factors login to view 2 more bullets Associated conditions login to view 15 more bullets Presentation Abdominal mass in previously healthy, asymptomatic child Symptoms (25-30% of children) login to view 5 more bullets Unusual presentation of clinical emergency login to view 2 more bullets Physical exam login to view 4 more bullets IMAGING Best initial test login to view 1 more bullet Most accurate test login to view 1 more bullet Radiography or CT to detect lung metastases STUDIES Tissue diagnosis after surgical resection login to view 3 more bullets Differential Diagnosis Neuroblastoma Polycystic kidney disease Other primary renal malignancies Treatment Prevention login to view 1 more bullet Standard therapy login to view 2 more bullets If with metastases login to view 1 more bullet Post-surgery login to view 1 more bullet Complications Nephropathy Hypertension Renal failure Prognosis Better prognosis with younger age Overall 5-year survival rate > 90%