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Familial pancreatic cancer: Concept, management and issues
- Source :
- World Journal of Gastroenterology
- Publication Year :
- 2017
- Publisher :
- Baishideng Publishing Group Inc., 2017.
-
Abstract
- Familial pancreatic cancer (FPC) is broadly defined as two first-degree-relatives with pancreatic cancer (PC) and accounts for 4%-10% of PC. Several genetic syndromes, including Peutz-Jeghers syndrome, hereditary pancreatitis, hereditary breast-ovarian cancer syndrome (HBOC), Lynch syndrome, and familial adenomatous polyposis (FAP), also have increased risks of PC, but the narrowest definition of FPC excludes these known syndromes. When compared with other familial tumors, proven genetic alterations are limited to a small proportion (< 20%) and the familial aggregation is usually modest. However, an ethnic deviation (Ashkenazi Jewish > Caucasian) and a younger onset are common also in FPC. In European countries, “anticipation” is reported in FPC families, as with other hereditary syndromes; a trend toward younger age and worse prognosis is recognized in the late years. The resected pancreases of FPC kindred often show multiple pancreatic intraepithelial neoplasia (PanIN) foci, with various K-ras mutations, similar to colorectal polyposis seen in the FAP patients. As with HBOC patients, a patient who is a BRCA mutation carrier with unresectable pancreatic cancer (accounting for 0%-19% of FPC patients) demonstrated better outcome following platinum and Poly (ADP-ribose) polymerase inhibitor treatment. Western countries have established FPC registries since the 1990s and several surveillance projects for high-risk individuals are now ongoing to detect early PCs. Improvement in lifestyle habits, including non-smoking, is recommended for individuals at risk. In Japan, the FPC study group was initiated in 2013 and the Japanese FPC registry was established in 2014 by the Japan Pancreas Society.
- Subjects :
- Oncology
medicine.medical_specialty
medicine.medical_treatment
Pancreatic Intraepithelial Neoplasia
Antineoplastic Agents
Review
familial pancreatic cancer
high risk
registry
Familial adenomatous polyposis
Cancer syndrome
03 medical and health sciences
Pancreatectomy
0302 clinical medicine
Japan
Risk Factors
Internal medicine
Pancreatic cancer
medicine
Humans
Genetic Predisposition to Disease
Early Detection of Cancer
BRCA2 Protein
Hereditary pancreatitis
BRCA1 Protein
business.industry
Incidence
Carcinoma
Gastroenterology
General Medicine
medicine.disease
United States
Lynch syndrome
Europe
Pancreatic Neoplasms
medicine.anatomical_structure
030220 oncology & carcinogenesis
Mutation
surveillance
030211 gastroenterology & hepatology
genetic
Pancreas
business
Subjects
Details
- Language :
- English
- ISSN :
- 10079327
- Volume :
- 23
- Issue :
- 6
- Database :
- OpenAIRE
- Journal :
- World journal of gastroenterology
- Accession number :
- edsair.doi.dedup.....a7f06328364c97fada70dac3055ed84f