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Inherited Pancreatic Cancer Syndromes

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Pancreatic Cancer

Part of the book series: M. D. Anderson Solid Tumor Oncology Series ((MDA))

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Summary

As we have documented, up to 10% of cases of pancreatic cancer occur as a result of an inherited genetic predisposition. This incidence of genetic susceptibility is higher than reported for all other inherited cancer syndromes. Furthermore, given that the penetrance for the genes identified to date is low and the development of pancreatic cancer in affected individuals occurs later in life, the 10% estimate for an inherited basis of pancreatic cancer is low. In considering the true frequency for an inherited predisposition to pancreatic cancer it is important to take into account the fact that all the genetic changes associated with pancreatic adenocarcinoma detailed in this review have been identified within the past 10 years.

Knowing that the incidence of an inherited basis for pancreatic cancer is high and that results for standard treatment are poor, the question arises of whether we can use our current knowledge to screen and prophylactically treat patients at risk. Unlike with colorectal cancer and breast cancer, there is currently no simple, reliable test to screen patients for pancreatic cancer. Additionally, the required prophylactic surgery for pancreatic cancer is associated with a significantly higher morbidity and mortality than that required for patients with a genetic predisposition for breast and colorectal cancer. At this time it would be unwise to recommend intensive screening of any single group for pancreatic cancer, with the possible exception of patients with hereditary pancreatitis who have developed chronic changes in their pancreas. In this small subset of patients a relative risk of 40 to 75 times the general population may justify screening.

Further research into the pathogenesis of pancreatic cancer will undoubtedly uncover other genes that may confer an increased risk for this disease. When this research is coupled with work aimed at understanding the mechanism by which the currently known genes contribute to pancreatic carcinogenesis, we hope to make a significant impact on the mortality of this disease.

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Authors
  1. David H. Berger
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  2. William E. Fisher
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Editors and Affiliations

  1. Department of Surgical Oncology, The University of Texas M.D. Anderson Cancer Center, Houston, TX, 77030, USA

    Douglas B. Evans M.D. (Professor of Surgery) & Peter W. T. Pisters M.D., F.A.C.S. (Associate Professor of Surgery) (Professor of Surgery) &  (Associate Professor of Surgery)

  2. Department of Gastrointestinal Medical Oncology, The University Texas M.D. Anderson Cancer Center, Houston, TX, 77030, USA

    James L. Abbruzzese M.D. (Professor of Medical Oncology, Chairman) (Professor of Medical Oncology, Chairman)

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© 2002 Springer-Verlag New York, Inc.

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Berger, D.H., Fisher, W.E. (2002). Inherited Pancreatic Cancer Syndromes. In: Evans, D.B., Pisters, P.W.T., Abbruzzese, J.L. (eds) Pancreatic Cancer. M. D. Anderson Solid Tumor Oncology Series. Springer, New York, NY. https://doi.org/10.1007/0-387-21600-6_6

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  • DOI: https://doi.org/10.1007/0-387-21600-6_6

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-0-387-95185-0

  • Online ISBN: 978-0-387-21600-3

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