| Familial pancreatic carcinoma in Jews. | |
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MedLine Citation:
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PMID: 15516847 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Pancreatic cancer (PC) is the most fatal of all gastrointestinal cancers, wherein its mortality compares strikingly with its incidence. Unfortunately, 80-90% of PCs are diagnosed in the nonresectable stage. While the lifetime risk of PC in developed countries is approximately 1-3%, it is the fifth most common cause of cancer deaths among both males and females in Western countries. It occurs in excess in Jews. Approximately 5-10% of PC shows familial clustering. Examination of such familial clusters must take into consideration cancers of diverse anatomic sites, such as malignant melanoma in the familial atypical multiple melanoma (FAMMM) syndrome due to the CDKN2A (p16) germline mutation, and combinations of colorectal and endometrial carcinoma, ovarian carcinoma, and several other cancers in hereditary nonpolyposis colorectal cancer (HNPCC), which are due to mismatch repair germline mutations, the most common of which are MSH2 and MLH1 . Other hereditary disorders predisposing to PC include Peutz-Jeghers syndrome, due to the STK11 mutation, familial pancreatitis due to the cationic trypsinogen gene, site-specific familial pancreatic cancer which may be due to the 4q32-34 mutation, hereditary breast-ovarian cancer (HBOC) syndrome that is due to BRCA2 and possibly some families with HBOC that is due to BRCA1 , familial adenomatous polyposis due to the ATP gene, and ataxia telangiectasia due to the ATM germline mutation. This extant heterogeneity mandates that the physician be knowledgeable about these PC-prone syndromes which play such an important role when considering the differential diagnosis of hereditary PC. Unfortunately, there are no PC screening programs with acceptable sensitivity and specificity. However, the gold standard for screening at this time is endoscopic ultrasound. Clearly, there is a great need for the development of novel screening approaches with acceptable sensitivity and specificity. Further research is needed to elucidate those etiologic factors that contribute to the apparent excess of PC in Ashkenazi Jews. Attention should also be given to the search for mutations predisposing to PC in Jews so that opportunities to learn more about the disease's pathogenesis, as well as screening and control, may take place. |
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Authors:
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Henry T Lynch; Carolyn A Deters; Jane F Lynch; Randall E Brand |
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Publication Detail:
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Type: Comparative Study; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Review |
Journal Detail:
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Title: Familial cancer Volume: 3 ISSN: 1389-9600 ISO Abbreviation: Fam. Cancer Publication Date: 2004 |
Date Detail:
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Created Date: 2004-11-01 Completed Date: 2005-02-15 Revised Date: 2007-11-14 |
Medline Journal Info:
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Nlm Unique ID: 100898211 Medline TA: Fam Cancer Country: Netherlands |
Other Details:
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Languages: eng Pagination: 233-40 Citation Subset: IM |
Affiliation:
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Department of Preventive Medicine and Public Health, Creighton University School of Medicine, 2500 California Plaza, Omaha, NE 68131, USA. htlynch@creighton.edu |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Age Distribution Aged Carcinoma, Pancreatic Ductal / ethnology, genetics*, pathology Female Genes, p16 Genetic Predisposition to Disease / ethnology* Germ-Line Mutation Humans Incidence Jews / genetics* Male Middle Aged Neoplastic Syndromes, Hereditary / ethnology*, genetics, pathology Pancreatic Neoplasms / ethnology, genetics*, pathology Pedigree Risk Assessment Sex Distribution Survival Analysis United States / epidemiology |
| Grant Support | |
ID/Acronym/Agency:
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1U01CA86389/CA/NCI NIH HHS |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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