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Early Diagnosis and Treatment of Pancreatic Dysplasia in Patients with a Family History of Pancreatic Cancer

Teresa A. Brentnall, MD; Mary P. Bronner, MD; David R. Byrd, MD; Rodger C. Haggitt, MD; and Michael B. Kimmey, MD
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From University of Washington, Seattle, Washington.

Requests for Reprints: Teresa A. Brentnall, MD, Division of Gastroenterology, Box 356424, University of Washington, Seattle, WA 98195; e-mail, teribr@u.washington.edu.

Current Author Addresses: Drs. Brentnall and Kimmey: Division of Gastroenterology, Box 356424, University of Washington, Seattle, WA 98195.

Drs. Bronner and Haggitt: Department of Pathology, Box 356100, University of Washington, Seattle, WA 98195.

Dr. Byrd: Department of Surgery, Box 356410, University of Washington, Seattle, WA 98195.

Ann Intern Med. 1999;131(4):247-255. doi:10.7326/0003-4819-131-4-199908170-00003
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Pancreatic cancer is the fourth leading cause of cancer death in the United States, and its incidence is increasing. At the time of diagnosis, 96% to 99% of patients are incurable and have a median survival of less than 1 year (13). Hereditary predisposition accounts for at least 10% of cases of pancreatic cancer (4). Some inherited syndromes that confer a high risk for pancreatic cancer include familial adenomatous polyposis, hereditary nonpolyposis colorectal cancer, cystic fibrosis, and hereditary pancreatitis. However, patients who inherit pancreatic cancer in an autosomal dominant fashion (familial pancreatic cancer) are at the highest risk, which can approach 50% (45).

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Figure 1.
Families X, Y, and Z.

CA = cancer; CHF = congestive heart failure; d. = died; DM = diabetes mellitus; MI = myocardial infarction; MVA = motor vehicle accident; SIDS = sudden infant death syndrome.

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Figure 2.
Endoscopic ultrasonography image from a patient with widespread dysplasia (patient Z.V.3).arrowsarrowheads

Hypoechoic nodules ( ) and echogenic foci ( ) are visible in the pancreatic tail.

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The dilated, irregular main duct with large side-branch sacculations and irregularities demonstrate a range of subtle to striking changes.
Figure 3. Endoscopic retrograde cholangiopancreatography image from patient X.III.17.
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Figure 4.
Histologic features of the pancreas in patient Z.V.3, showing multifocal low-grade dysplasia (atypical ductal hyperplasia).

Low-grade dysplastic epithelium with stratified, enlarged, hyperchromatic nuclei are adjacent to ductal epithelium that are normal in appearance.

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