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Clin Transl Oncol ; 14(8): 553-63, 2012 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-22855135

RESUMO

Pancreatic carcinoma (PC) represents the fourth leading cause of cancer death in Spain with a death rate of 2,400 males and 2,000 females per year. Poor outcome related to its silent nature and the lack of reliable secondary prevention measures translate into advanced-stage diagnosis, 75 % of deaths within the first year of diagnosis and 5-year survival rate of <5 %. Family history was first recognized as a risk factor for PC. Further population-based and case-control studies subsequently found that 7.8 % of patients with PC have a family history of the same tumor and individuals with a first-degree relative with PC have a 3.2-fold increased risk of developing PC. Overall, it is estimated that up to 10 % of PC have a familial component. However, known genetic syndromes account for <20 % of the observed familial aggregation of PC. We review the most important aspects in epidemiology, molecular biology and clinical management of familial PC.


Assuntos
Carcinoma/diagnóstico , Carcinoma/genética , Pâncreas/patologia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/genética , Carcinoma/patologia , Feminino , Predisposição Genética para Doença , Humanos , Masculino , Programas de Rastreamento , Neoplasias Pancreáticas/patologia , Guias de Prática Clínica como Assunto , Lesões Pré-Cancerosas/diagnóstico , Lesões Pré-Cancerosas/genética , Lesões Pré-Cancerosas/patologia , Sistema de Registros , Fatores de Risco , Taxa de Sobrevida
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