Familial pancreatic adenocarcinoma in three generations. A case report and a review of the literature

Ehrenthal, Deborah B.; Haeger, Lisa; Griffin, Thomas J.; Compton, Carolyn C.

doi:10.1002/1097-0142(19870501)59:9<1661::aid-cncr2820590923>3.0.co;2-h

Cited by 70 publications

(25 citation statements)

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“…Familial aggregation of pancreas cancer has been reported in various populations [8][9][10][11] , including the families in this current study 12 . Familial clustering of pancreas or other cancers may be influenced by shared environmental factors and by shared genes.…”

Section: Introductionmentioning

confidence: 85%

Risk of Other Cancers in Individuals with a Family History of Pancreas Cancer

Coté

Schenk

Schwartz

et al. 2007

J Gastrointest Canc

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Background-Inherited predisposition to pancreas cancer accounts for approximately 10% of cases. Familial aggregation may be influenced by shared environmental factors and shared genes. We evaluate whether a family history of pancreas cancer is a risk factor for ten specified cancers in first-degree relatives: bladder, breast, colon, head & neck, lung, lymphoma, melanoma, ovary, pancreas and prostate.

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Section: Introductionmentioning

confidence: 85%

Risk of Other Cancers in Individuals with a Family History of Pancreas Cancer

Coté

Schenk

Schwartz

et al. 2007

J Gastrointest Canc

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“…Although about 90% of pancreatic cancer patients report a negative family history of pancreatic cancer, familial clustering in the remaining 5% to 10% of the disease (presumably attributed to inheritance of moderate or high penetrance genes) has been reported (5)(6)(7)(8)(9). Formal case-control investigations provide persuasive evidence that a family history of pancreatic cancer is a risk factor, and some have further elaborated the role of cigarette smoking or age at diagnosis of pancreatic cancer in the family setting.…”

Section: Introductionmentioning

confidence: 99%

Pancreatic Cancer Genetic Epidemiology Consortium

Petersen

Andrade

Goggins

et al. 2006

Cancer Epidemiology, Biomarkers &Amp; Prevention

134

106

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We have organized the Pancreatic Cancer Genetic Epidemiology (PACGENE) Consortium to identify susceptibility genes in familial pancreatic cancer (FPC). The Consortium comprises seven data collection centers, a statistical genetics core, and a pathology/archival genotyping core. We recruit kindreds containing two or more affected blood relatives ascertained through incident pancreatic adenocarcinoma cases, physician referrals, and/or through Internet recruitment. Accrual to a database containing core clinical, demographic, lifestyle, and family history information from questionnaires is ongoing, along with biospecimen collection. To date, 13,147 patients have been screened for family history, of whom 476 (50% male) probands and 1,912 of their adult (99% unaffected) relatives have been enrolled. Of these, 379 kindreds meet criteria for FPC, having at least two first-degree relatives with pancreatic cancer. Cumulative incidence curves using available age of diagnosis (onset) among and affected relatives were compared with those for incident pancreatic cancer cases reported to 13 U.S. Surveillance Epidemiology and End Results (SEER) sites from 1973 to 2000 (N = 72,700). The mean age F SD at diagnosis among 466 PACGENE probands and 670 affected relatives was 64.1 F 11.8 and was 65.4 F 11.6 for the subset of 369 FPC probands and 429 relatives. Both samples were significantly younger than the mean age at diagnosis in the SEER population (70.0 F 12.1 years; differences in curves versus SEER, P < 0.001). Age at diagnosis (excluding probands) in FPC kindreds does not decrease with increasing number of affected individuals. In our sample, younger age at diagnosis was observed whether we grouped probands by recruitment sites that predominantly recruited through high-risk referrals, or through screening all pancreatic cancer patients for family history. Linkage studies are ongoing. The PACGENE Consortium will be a valuable familybased resource that will greatly enhance genetic epidemiology research in pancreatic cancer. (Cancer Epidemiol Biomarkers Prev 2006;15(4):704 -10)

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“…4 Enstrom found that the mor tality from pancreatic cancer among Mor mons in California and Utah was less than that for nonsmoking white males according to data obtained from the National Center for Health Statistics.5 Familial occurrence of pancreatic cancer is rare, but a few re ports have appeared; a familial tendency has not been well documented, however. 6 An apparent association between dia betes and pancreatic cancer has interested investigators for many years. While it is not unusual for diabetes to precede the diagnosis of pancreatic cancer by several months, there does not seem to be a causal relationship, at least in males.…”

Section: Epidemiologymentioning

confidence: 99%