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01-08-2012 | Educational Series—BLUE SERIES

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

Authors: P. Pérez Segura, C. Guillén Ponce, T. Ramón y Cajal, R. Serrano Blanch, E. Aranda

Published in: Clinical and Translational Oncology | Issue 8/2012

Abstract

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.

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Title: Hereditary pancreatic cancer: molecular bases and their application in diagnosis and clinical management. A guideline of the TTD group
Authors: P. Pérez Segura
C. Guillén Ponce
T. Ramón y Cajal
R. Serrano Blanch
E. Aranda
Publication date: 01-08-2012
Publisher: Springer Milan
Published in: Clinical and Translational Oncology / Issue 8/2012
Print ISSN: 1699-048X
Electronic ISSN: 1699-3055
DOI: https://doi.org/10.1007/s12094-012-0840-0

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