caa a22 4500 445889314 CHVBK 20180317145610.0 cr unu---uuuuu 170323e20111101xx s 000 0 eng 10.1007/s00535-011-0457-z doi (NATIONALLICENCE)springer-10.1007/s00535-011-0457-z Matsubayashi Hiroyuki Division of Endoscopy, Shizuoka Cancer Center, 1007 Shimonagakubo, Nagaizumi, Suntogun, 411-8777, Shizuoka, Japan aut Familial pancreatic cancer and hereditary syndromes: screening strategy for high-risk individuals [Elektronische Daten] [Hiroyuki Matsubayashi] Globally, and almost evenly across nations, a familial disposition can be found in 4-10% of patients with pancreatic cancer (PC). A family history of PC is a risk for this disease and the risk level changes in correlation with the number of affected relatives. Several hereditary syndromes with potential germline mutation also have a high risk for PC; however, little is yet known regarding the genes responsible for familial pancreatic cancer (FPC). Characteristics of FPC cases are similar to those of other familial tumors, including younger onset than in sporadic cases and an ethnic difference (Ashkenazi Jewish>other Caucasian). Other risks resemble those of sporadic cases and include smoking and diabetes mellitus. People with several genetic syndromes, including Peutz-Jeghers syndrome, hereditary pancreatitis, breast-ovarian cancer syndrome, hereditary nonpolyposis colorectal cancer, and familial adenomatous polyposis also have an increased risk of PC. In many countries, but not yet in Japan, screening of these high-risk individuals is now ongoing for the detection of early PC under established familial pancreatic cancer registries. In addition to the ordinary risk factors, such as smoking, diabetes, pancreatitis, cysts, duct ectasia, and intraductal papillary mucinous neoplasm (IPMN), individuals with a family history of PC and hereditary syndromes are expected to be entered into the screening protocol. Springer, 2011 Pancreatic cancer nationallicence Family history nationallicence Hereditary syndrome nationallicence Risk factor nationallicence Screening nationallicence PC : Pancreatic cancer nationallicence SIR : Standardized incidence rate nationallicence OR : Odds ratio nationallicence IPMN : Intraductal papillary mucinous neoplasm nationallicence FPC : Familial pancreatic cancer nationallicence FDR : First-degree relative nationallicence PJS : Peutz-Jeghers syndrome nationallicence FAMMM : Familial atypical multiple mole melanoma nationallicence HBOC : Hereditary breast-ovarian cancer nationallicence RR : Relative risk nationallicence PACGENE : Pancreatic Cancer Genetic Epidemiology Consortium nationallicence NFPTR : National Familial Pancreas Tumor Registry nationallicence SPC : Sporadic pancreatic cancer nationallicence EUROPAC : European Registry of Hereditary Pancreatitis and Familial Pancreatic Cancer nationallicence FaPaCa : German National Case Collection for Familial Pancreatic Carcinoma nationallicence HNPCC : Hereditary nonpolyposis colorectal cancer nationallicence FAP : Familial adenomatous polyposis nationallicence MSI : Microsatellite instability nationallicence PanIN : Pancreatic intraepithelial neoplasia nationallicence PanScan : Pancreatic Cancer Cohort Consortium nationallicence EUS : Endoscopic ultrasonography nationallicence CT : Computed tomography nationallicence MRI : Magnetic resonance imaging nationallicence MRCP : Magnetic resonance cholangiopancreatography nationallicence ERCP : Endoscopic retrograde cholangiopancreatography nationallicence MCN : Mucinous cystic neoplasm nationallicence Journal of Gastroenterology Springer Japan 46/11(2011-11-01), 1249-1259 0944-1174 46:11<1249 2011 46 535 https://doi.org/10.1007/s00535-011-0457-z text/html Onlinezugriff via DOI 1 review-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00535-011-0457-z text/html Onlinezugriff via DOI NATIONALLICENCE 100 1- Matsubayashi Hiroyuki Division of Endoscopy, Shizuoka Cancer Center, 1007 Shimonagakubo, Nagaizumi, Suntogun, 411-8777, Shizuoka, Japan aut NATIONALLICENCE 773 0- Journal of Gastroenterology Springer Japan 46/11(2011-11-01), 1249-1259 0944-1174 46:11<1249 2011 46 535 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer