Development of a Registry for Familial Pancreatic Cancer

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DESCRIPTION (provided by applicant): Adenocarcinoma of the pancreas is the fourth most common cause of cancer death in the United States. It affects about 28,600 Americans each year and the mortality rate equals the incidence. Five to 10 percent of cases aggregate in families generally in a pattern consistent with autosomal dominant transmission with variable penetrance. There are a few rare syndromes for which a genetic etiology has been identified. However, the etiology has not been defined for the majority of familial cases. Identification of high-risk families and registration of the members is fundamental as a resource for gene discovery and future studies to refine identification of environmental factors, develop prevention strategies and test interventions. The aim of this proposal is to develop a high-risk population registry to provide a basis for future research and the specific objectives are: 1) Characterize the families with clustering of pancreatic cancer identified in the Utah Population Database (UPDB), 2) Create a registry of these families, 3) Procure blood and tissue blocks for DNA extraction, 4) Establish systems necessary so that the persons in this registry are available to participate in future studies to include gene discovery, identification and validation of environmental and lifestyle risk factors, chemoprevention trials, and interventional studies that potentially could include medical (targeted gene therapy, tumor vaccines), surgical and endoscopic modalities. The investigators intend to participate in an international consortium of registries currently in development to increase the statistical power for such studies as listed above. Established and validated genetic epidemiological techniques developed at this institution will be used to characterize the families. Probands, or next of kin for deceased persons, from informative families will be contacted and invited to participate using a validated method that assures confidentiality. For consenting families, a family advocate will be identified to recruit relatives and assist in refining the pedigree. Consent will be obtained to collect medical records and pancreatic cancer tissue blocks from the proband. The pedigrees will be expanded and registered. Subjects will be notified of future registry studies to include questionnaire completion, blood testing for DNA, and clinical trials to be developed. This proposal will allow the investigators to provide access a unique and important resource on a local and a national level. The goal corresponds with the National Cancer Institute Initiative to "identify, characterize, and understand the genes that cause or play a role in familial cancer."