Defining an optimal time window to screen for hepatoblastoma in children with Beckwith-Wiedemann syndrome.

Patients with Beckwith-Wiedemann spectrum (BWSp) undergo quarterly alpha-fetoprotein measurement for hepatoblastoma (HB) screening up to 4 years of age, paralleling the epidemiology of nonsyndromic HB. However, specific data on the timing of HB development in BWSp are lacking. Here we compare the timing of presentation of HBs in BWSp with a control cohort of consecutive HB cases, demonstrating that halving screening duration of screening procedures in BWSp likely will not impact its effectiveness.