Hereditary pancreatic cancer: molecular bases and their application in diagnosis and clinical management. A guideline of the TTD group
Por:
Segura, PP, Ponce, CG, Cajal, TRY, Blanch, RS, Aranda, E
Publicada:
1 ago 2012
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.
Filiaciones:
Segura, PP:
Clin Hosp San Carlos, Madrid 28046, Spain
Ponce, CG:
Hosp Ramon & Cajal, Madrid, Spain
Cajal, TRY:
Hosp San Pau, Barcelona, Spain
Blanch, RS:
Hosp Reina Sofia, Cordoba, Spain
Aranda, E:
Hosp Reina Sofia, Cordoba, Spain
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