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SÃndrome de Li-Fraumeni
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Definição:
Rare autosomal dominant syndrome characterized by mesenchymal and epithelial neoplasms at multiple sites. of the p53 tumor suppressor gene, a component of the 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 and soft tissue sarcomas (); ; ; ; and .
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