Wilms Tumor: An Example Of Risk-Adapted and Well Tolerated Therapy

Abstract

Wilms tumor (WT) is the most common malignant renal tumor in children. It can present with an abdominal mass and macroscopic hematuria. A 2 year old boy presented with acute onset of bilious vomiting and macroscopic hematuria. He was diagnosed with Stage 4 WT with lung metastasis and treated with risk-adapted therapy. Initial surgery was followed by risk based chemotherapy; lung radiation was needed and he currently remains in remission. Due to the recent advances in treatment based on risk stratification of WT, the overall survival for children diagnosed with WT has increased, but treatment of lung metastases can be challenging.

Wilms tumor presenting as small bowel obstruction in a neonate: A diagnostic challenge

“…Wilms tumor, also called nephroblastoma, is the commonest primary malignant renal tumor in children accounting for 6% of overall childhood malignancies and more than 90% of pediatric renal tumors [5] , [6] , [7] , [8] . Most (~95%) cases of Wilms tumors are sporadic and few cases may occur in association with other congenital malformations such as aniridia, hemihypertrophy, cryptorchidism, hypospadias, gonadal dysgenesis, pseudohermaphroditism, and horseshoe kidney, or genetic syndromes such as Beckwith-Wiedemann syndrome, Denys-Drash syndrome, and WAGR syndrome (Wilms tumor, aniridia, ambiguous genitalia, mental retardation) [9] , [10] , [11] , [12] .…”