WILMS TUMORMost common renal malignancy in children <15yomedian age at diagnosis: 43 months in girls and 37 months in boys

ASSOCIATED WITH- AniridiaGenitourinary abnormalities- Loss of function mutations of tumor suppressor and transcription genes

CLINICAL PRESENTATIONMost common: Asymptomatic abdominal mass or swelling (typically found by parents while bathing child)- Abdominal pain (30-40%)- Hematuria (12-25%)- Hypertension (25%)

PHYSICAL EXAMCareful!!! Vigorous palpation may rupture the renal capsule, resulting in tumor spillage, increasing the tumor stage and the need for more intensive therapy- firm, non-tender, smooth mass- eccentrically located - rarely crosses the midline

DIAGNOSIS1.      Abdominal ultrasound with evaluation of mass (assess flow)2.      CT/MRI abdomen can be used to further evaluate the lesion3.      Routine CT chest - Lung is the most common metastatic site4.      Definitive: biopsy with histologic confirmation

STAGINGBased on anatomic extent of tumor

MANAGEMENTRefer to a pediatric cancer centerTypically multimodal therapy involving chemotherapy, radiation therapy and surgery    -Surgical excision for respectable tumors    -Chemotherapy for all patients except extremely low risk    -Radiation therapy indicated by stage and histology

Overall five-year survival for Wilms tumor is 90%