Wilms tumor is one of the most common abdominal tumors seen in the pediatric population. It is of embryonal origin and thought to be due to loss of function in the tumor suppressor genes WT1 and WT2. It is associated with Beckwith-Weidemann syndrome which has symptoms of macroglossia, hemihyperplasia, and organomegaly. Wilms tumor also has an association with WAGR syndrome (Wilms tumor, Aniridia, Genitourinary anomalies, and Retardation). 

This patient presented with a very large Wilms tumor in the superior pole of the right kidney with extensive nodules indicating pulmonary metastasis. Treatment for Wilms tumor includes nephrectomy and chemotherapy. In the case of metastasis such as this case, radiation is added as adjuvant therapy. Prognosis is generally very good for Wilms tumor (>90% survival), however, patients fare worse with metastasis then with local disease, unfortunately.