Wilms Tumor (Nephroblastoma)

The tumor is well-demarcated and has soft, pink-tan cut surface bulging into the renal pelvis. In addition to WAGR syndrome and Denys-Drash syndrome, the patients with Beckwith-Wiedemann syndrome (BWS) are at increased risk of developing Wilms tumor. This syndrome is characterized by organomegaly, hemihypertrophy, macroglossia, omphalocele, adrenal cytomegaly, and increased risk of developing Wilms tumor. BWS is linked to loss of genomic imprinting at the WT2 locus on band 11p15.5. A number of different genes in this region, including IGF2, have been implicated in different cases of BWS. Image courtesy of Dr. Jean-Christophe Fournet, Paris, France; humpath.com; Used with permission