Article history: Received 28 June 2016 Accepted 28 June 2016 Available online 4 July 2016 pathway) and genetic progression (e.g., the Fearon-Vogelstein pathway Fearon and Vogelstein, 1990). Steps in adult tumor pathogenesis was suggested by Armitage and Doll 60 years ago (Armitage and Doll, 1954), with around 7 steps. The question then for pediatric tumors, is how do steps occur at such a young...

BACKGROUND Xanthogranulomatous pyelonephritis (XGP) is a rare renal tumor that arises as a complication of chronic obstructive pyelonephritis of uncertain etiology. It is primarily an adult tumor seen occasionally in children associated with urinary tract obstruction due to congenital urological anomalies, nephrolithiasis, and recurrent urinary tract infections. Radiologically, it may show neop...

On the search for a genetic cause 46,XX virilization due to testicular (TDSD) or ovotesticular DSD (OTDSD), 78 individuals were studied by whole exome sequencing. In 7 cases, heterozygous de novo variants found in 4th zinc finger (ZF4) of Wilms tumor 1 gene (WT1). Modelling these human granulosa cells and mice recapitulated phenotype.

BACKGROUND Children with overgrowth syndromes including isolated hemihyperplasia have an increased risk for developing embryonal tumors, particularly Wilms' tumor and hepatoblastoma. Screening with sonography has been suggested as a method of identifying these tumors while they are still at an early stage. CASE PRESENTATION We describe an infant diagnosed with Wilms tumor in whom isolated hem...