Which tumors are patients with Beckwith-Wiedemann Syndrome (BWS) at significant risk for?

Patients with Beckwith-Wiedemann Syndrome (BWS) are at significant risk for developing Wilms tumor and hepatoblastoma. BWS is a genetic condition characterized by overgrowth and an increased risk of specific embryonal tumors due to abnormalities in imprinted genes on chromosome 11.

Wilms tumor, a type of kidney cancer, is among the most common tumors associated with BWS. Children with this syndrome are monitored closely for the emergence of Wilms tumor, especially during early childhood, as the incidence is notably elevated in these patients.

Hepatoblastoma, a liver tumor, is also commonly associated with BWS. This tumor typically occurs in younger children, often before the age of 5, and is also significantly linked to the overgrowth and dysregulation seen in this syndrome.

The other options, while they represent various types of tumors, do not have a direct established association with Beckwith-Wiedemann Syndrome in terms of increased risk to the same extent as Wilms tumor and hepatoblastoma. This distinction is crucial for screening and management of children diagnosed with BWS.