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Pancreatic Cancer pp 81–96Cite as

Hereditary Pancreatitis and Pancreatic Cancer

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

Hereditary pancreatitis (HP) is a rare disorder characterized by recurrent attacks of acute pancreatitis, chronic pancreatitis, and a high incidence of adenocarcinoma of the pancreas. Hereditary pancreatitis specifically refers to otherwise unexplained pancreatitis in an individual from a family in which the pancreatitis phenotype appears to be inherited through a disease-causing gene mutation that is expressed in an autosomal dominant pattern (1). Familial pancreatitis refers to pancreatitis from any cause that occurs in a family with an incidence greater than expected by chance alone, given the size of the family and incidence of pancreatitis within a defined population (1). HP typically presents in childhood with an attack of acute pancreatitis. Recurrent acute pancreatitis advances to become chronic pancreatitis in about 50% of patients (2, 3). Chronic pancreatitis is a progressive inflammatory process that destroys pancreatic function and results in the malabsorption of nutrients (exocrine failure) and diabetes mellitus (endocrine failure). Additional complications of chronic pancreatitis that are seen in many, but not all, patients include severe and persistent pain, strictures of the common bile duct, pancreatic pseudocysts, and pancreatic duct stones that may require interventional or surgical treatment. Of those patients who develop a chronic course, about 40% develop pancreatic cancer (PC) (4, 5).

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Authors and Affiliations

  1. Division of Gastroenterology, Hepatology and Nutrition, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA

    Julia B. Greer MD, MPH

  2. Department of Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA

    Narcis O. Zarnescu MD

  3. Departments of Medicine, Cell Biology & Physiology, and Human Genetics, University of Pittsburgh, USA

    David C. Whitcomb MD, PhD

  4. University of Pittsburgh Medical Center, University of Pittsburgh Cancer Institute, Pittsburgh, Pennsylvania, USA

    David C. Whitcomb MD, PhD

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  1. Julia B. Greer MD, MPH
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Editors and Affiliations

  1. University of California San Diego, La Jolla, CA, USA

    Andrew M. Lowy

  2. School of Medicine, Johns Hopkins University, Baltimore, MD, USA

    Steven D. Leach

  3. Wayne State University, Detroit, MI, USA

    Philip A. Philip

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Greer, J.B., Zarnescu, N.O., Whitcomb, D.C. (2008). Hereditary Pancreatitis and Pancreatic Cancer. In: Lowy, A.M., Leach, S.D., Philip, P.A. (eds) Pancreatic Cancer. M. D. Anderson Solid Tumor Oncology Series. Springer, Boston, MA. https://doi.org/10.1007/978-0-387-69252-4_6

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