FaCD Online Syndrome Fact Sheet

Last updated: 27 May 2009

Name: Biliary Tract Cancer, Familial Clustering of

Synonym: incl. Familial Gall Bladder cancer

Mode of Inheritance: multifact?

Tumor features

biliary tract cancer (incl. gallbladder)

Tumor features (possible)

pancreatic adenocarcinoma

Comment

A Spanish-Italian study demonstrated an association between a family history of gallbladder cancer and the development of this tumor in a relative (RR 13.9)[1]. Hemmini et al [2] studied the occurrence of cancer in relatives of patients with liver or biliary tract cancers. The authors demonstrated a high risk for familial gall bladder cancer (SIR 5.21 (95% CI 2.07-10.80)) and for familial hepatocellular cancer (SIR 4.69 (95% CI 1.48-11.04)). For gall bladder and hepatocellular cancer, maternal transmission appeared to be more prevalent. Gall bladder cancer was associated with pancreatic cancer (SIR 2.39 (95% CI 1.23-4.18)).
In a large study of Chinese patients, Hsing et al[3] observed that for gallbladder cancer, individuals with gallstones but without a family history of gallstones had a 21-fold risk (95% CI 14.8-30.1), while those with both gallstones and a positive family history had a 57-fold risk (95% CI 32.0-110.5) of developing gallbladder cancer.

Variants in a number of genes may moderately affect biliary tract cancer risk[4-7]. These genes are not listed separately in this file

References

[1] Fernandez E, La Vecchia C, D'Avanzo B, Negri E, Franceschi S. Family history and the risk of liver, gallbladder, and pancreatic cancer. Cancer Epidemiol Biomarkers Prev 1994; 3(3):209-212.
[2] Hemminki K, Li X. Familial liver and gall bladder cancer: a nationwide epidemiological study from Sweden. Gut 2003; 52(4):592-6.
[3] Hsing AW, Bai Y, Andreotti G, Rashid A, Deng J, Chen J, Goldstein AM, Han TQ, Shen MC, Fraumeni JF Jr, Gao YT. Family history of gallstones and the risk of biliary tract cancer and gallstones: a population-based study in Shanghai, China. Int J Cancer. 2007 Aug 15;121(4):832-8.
[4] Park SK, Andreotti G, Sakoda LC, Gao YT, Rashid A, Chen J, Chen BE, Rosenberg PS, Shen MC, Wang BS, Han TQ, Zhang BH, Yeager M, Chanock S, Hsing AW. Variants in hormone-related genes and the risk of biliary tract cancers and stones: a population-based study in China. Carcinogenesis. 2009 Apr;30(4):606-14.
[5] Andreotti G, Chen J, Gao YT, Rashid A, Chen BE, Rosenberg P, Sakoda LC, Deng J, Shen MC, Wang BS, Han TQ, Zhang BH, Yeager M, Welch R, Chanock S, Fraumeni JF Jr, Hsing AW. Polymorphisms of genes in the lipid metabolism pathway and risk of biliary tract cancers and stones: a population-based case-control study in Shanghai, China.Cancer Epidemiol Biomarkers Prev. 2008 Mar;17(3):525-34.
[6] Zhang M, Huang WY, Andreotti G, Gao YT, Rashid A, Chen J, Sakoda LC, Shen MC, Wang BS, Chanock S, Hsing Variants of DNA repair genes and the risk of biliary tract cancers and stones: a population-based study in China. Cancer Epidemiol Biomarkers Prev. 2008 Aug;17(8):2123-7.
[7] Hou L, Xu J, Gao YT, Rashid A, Zheng SL, Sakoda LC, Shen MC, Wang BS, Deng J, Han TQ, Zhang BH, Meyers DA, Fraumeni JF Jr, Hsing AW. CYP17 MspA1 polymorphism and risk of biliary tract cancers and gallstones: a population-based study in Shanghai, China. Int J Cancer. 2006 Jun 1;118(11):2847-53.