Included titles and symbols : Adrenocortical carcinoma, pediatric;
Description : Adrenocortical carcinoma (ADCC) is a rare but aggressive childhood tumor, representing
about 0.4% of childhood tumors, with a high incidence of associated tumors. ADCC occurs
with increased frequency in patients with the Beckwith-Wiedemann syndrome (130650)
and is a component tumor in Li-Fraumeni syndrome (LFS; 151623).;
Inheritance : Autosomal dominant;
Molecular basis : Caused by mutation in the tumor protein p53 gene (TP53, 191170.0040);