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Adrenocortical carcinoma, hereditary

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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).

OMIM

  • Mode of Inheritance

  • Autosomal dominant inheritance

VARIANTS

11

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Genes

External Links

  • OMIM

    202300

  • Orphanet
  • HPO
  • Medgen

    C1859972

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