Familial pancreatic carcinoma in Jews

Abstract

This article is based upon a literature overview of cancer in Jews. It involves a comparison of variation in incidence and prevalence rates between Jews and non-Jews. However, the reader must exercise a certain amount of skepticism when considering secular changes in cancer incidence and prevalence and the public health implications of such cancer variation. Ashkenazi Jews have a lifetime CRC risk of 9--15%. This elevated CRC risk is similar to that of individuals in the "familial risk'' category, and differs strikingly from the 5-6% CRC risk for non-Ashkenazi members of general Western populations. A MedLine search tested the hypothesis that site-specific and/or all-cancer incidence and mortality rates are either higher or lower than expected in Ashkenazi Jews worldwide, when compared with reference populations. Results showed that all cancer incidence and mortality is not higher in Ashkenazi Jews when compared to North American non-Hispanic whites. Indeed, rates for some cancers, such as carcinoma of the lung in Ashkenazi males, are low; this example is likely attributable in large part to decreased tobacco use. Carcinoma of the ovary, pancreas, stomach, and non-Hodgkin's lymphoma have a higher incidence rate in Ashkenazi. Even though BRCA1 and BRCA2 founder mutations which predispose to carcinoma of the breast and ovary appear increased in Ashkenazi breast cancer affected women, there was no evidence supporting an elevated risk of breast cancer among Ashkenazi women. Our primary concern, however, is that Ashkenazi Jews may have one of the highest lifetime CRC risks of any ethnic group in the world, a risk that diverges significantly from that of the general population; therein, it logically calls for more intensive CRC screening guidelines. We have emphasized that the reader use caution in the interpretation of statistics which portray variation in incidence and prevalence figures for cancer in any racial, ethnic, or religious group, inclusive, of course, of Jews. Clearly, more research will be required in the interest of accuracy in the understanding of these cancer variations, since they portend the need for special cancer control strategies. A lesser degree of attention can then be given to carcinoma of the penis and uterine cervix, which occur very infrequently in Jews. We urge our colleagues to continue to probe further into these statistical differences in cancer's incidence and prevalence in order to garner a better understanding of cancer's etiology and pathogenesis.

Pancreatic cancer and medical history in a population-based case–control study in the San Francisco Bay Area, California

“…While most of these cancers occur sporadically, individuals who harbor germline mutations predisposing them to these cancers have an increased risk of pancreatic cancer ranging from 3.5-to 22-fold [67][68][69][70][71]. However, we did not observe a statistically significant increased risk of pancreatic cancer in individuals who had had a prior history of any of these site-specific malignancies.…”

Familial pancreatic cancer and hereditary syndromes: screening strategy for high-risk individuals

“…A family history of PC is another risk factor [7][8][9][10][11][12][13][14][15][16]. Familial PC (FPC), which is defined as the presence of two first-degree relatives (FDRs) with PC and is not associated with known hereditary syndromes, accounts for 4-10% of all PC cases [7] (similar incidences in western countries and Japan [17]) ( Table 1) [17][18][19][20][21][22][23][24][25][26][27][28].…”

“…Familial PC (FPC), which is defined as the presence of two first-degree relatives (FDRs) with PC and is not associated with known hereditary syndromes, accounts for 4-10% of all PC cases [7] (similar incidences in western countries and Japan [17]) ( Table 1) [17][18][19][20][21][22][23][24][25][26][27][28]. Some inherited syndromes also show higher risks, such as Peutz-Jeghers syndrome (PJS) [29], hereditary pancreatitis [30], familial atypical multiple mole melanoma (FAMMM), and hereditary breast-ovarian cancer (HBOC) [11] (Table 2).…”

“…The incidence of PC increases with the number of family members with PC, so that a 4.5 times increased risk of PC occurs for persons with one FDR with PC, with the increased risk being 6.4 times with two FDRs, and up to 32 times with three FDRs [9], although families with C3 PC cases within FDRs are rare in Japan (0.5%) [17]. Smoking (especially current smoking) [17,33], diabetes (recent onset of diabetes) [17], and ethnicity (Ashkenazi Jewish) [11] are also risks for FPC. The lifetime risk of PC in FPC kindreds also increases with decreasing age of onset of PC [34,35].…”

Werner syndrome as a hereditary risk factor for exocrine pancreatic cancer

“…[66][67][68][69][70][71][72][73][74][75][76][77][78][79][80][81][82][83][84] As shown in the clinical case series in this report, pancreatic adenocarcinoma in patients with WS is rare, but increasingly being recognized ( Table 1). We propose that WS is a heritable disease that predisposes affected individuals to develop pancreatic adenocarcinoma (Table 3).…”