" /> Adrenocortical carcinoma, hereditary - CISMeF





Preferred Label : Adrenocortical carcinoma, hereditary;

Symbol : ADCC;

CISMeF acronym : ADCC;

Type : Phenotype, molecular basis known;

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);

Neoplasia : Adrenocortical carcinoma;

Prefixed ID : #202300;

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16/05/2024


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