Hereditary pancreatic cancer: molecular bases and their application in diagnosis and clinical management. A guideline of the TTD group

• Autores: Pedro Pérez Segura, Carmen Guillén Ponce, Teresa Ramón y Cajal, Raquel Serrano Blanch, Enrique Aranda Aguilar
• Localización: Clinical & translational oncology, ISSN 1699-048X, Vol. 14, Nº. 8, 2012, págs. 553-563
• Idioma: inglés
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• Resumen

  • 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.