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

  • Educational Series—BLUE SERIES
  • Advances in Translational Oncology
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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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The authors declare that they have no conflict of interest related to the publication of this article.

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Authors and Affiliations

  1. Medical Oncology, Clinical Hospital San Carlos, 28046, Madrid, Spain

    P. Pérez Segura

  2. Medical Oncology, Hospital Ramón and Cajal, Madrid, Spain

    C. Guillén Ponce

  3. Medical Oncology, Hospital San Pau, Barcelona, Spain

    T. Ramón y Cajal

  4. Medical Oncology, Hospital Reina Sofía, Córdoba, Spain

    R. Serrano Blanch & E. Aranda

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  1. P. Pérez Segura
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  2. C. Guillén Ponce
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Correspondence to P. Pérez Segura.

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Segura, P.P., Ponce, C.G., Ramón y Cajal, T. et al. Hereditary pancreatic cancer: molecular bases and their application in diagnosis and clinical management. A guideline of the TTD group. Clin Transl Oncol 14, 553–563 (2012). https://doi.org/10.1007/s12094-012-0840-0

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