A Late Onset of Adrenocortical Cancer Assosiated with Beckwith-Wiedemann Syndrome

Abstract

Beckwith-Wiedemann syndrome (BWS) is a genetic overgrowth disorder involving a predisposition to tumor development. The common features of Beckwith-Wiedemann syndrome include omphalocele, macroglos- sia and macrosomia. The increased risk for neoplasia is concentrated in the first eight years of life. However, this case presents a late onset of adrenocortical cancer assosiated with Beckwith-Wiedemann syndrome.

Keywords

• beckwith-wiedemann syndrome
• hemihyperplasia
• adrenal tumors
• adrenal cortical cancer
• hyperandrogenia