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Concept information

Preferred term

Li-Fraumeni Syndrome  

Type

  • mesh:Descriptor

Definition

  • Rare autosomal dominant syndrome characterized by mesenchymal and epithelial neoplasms at multiple sites. MUTATION of the p53 tumor suppressor gene, a component of the DNA DAMAGE response pathway, apparently predisposes family members who inherit it to develop certain cancers. The spectrum of cancers in the syndrome was shown to include, in addition to BREAST CANCER and soft tissue sarcomas (SARCOMA); BRAIN TUMORS; OSTEOSARCOMA; LEUKEMIA; and ADRENOCORTICAL CARCINOMA.

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URI

http://data.loterre.fr/ark:/67375/JVR-JG6HVVK8-P

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RDF/XML TURTLE JSON-LD Created 6/21/91, last modified 6/29/18