FaCD Online Syndrome Fact Sheet

Last updated: 05 Aug 2008

Name: Biliary Tract Cancer, Multiple Primary Malignancies in Patients with

Tumor features

biliary tract cancer (incl. gallbladder)
cervical cancer
colorectal cancer
ovarian cancer (i.e. epithelial origin)

Tumor features (possible)

breast cancer
neuroendocrine (Merkel cell) skin carcinoma
pancreatic adenocarcinoma
testicular germ cell tumor


Su et al.[1] used SEER data to study associations of primary biliary tract cancer with other primary cancers. The relative risk (RR) of biliary tract cancer following cervical cancer was increased (2.04, 95% CI 1.02-3.64). In the reverse direction, i.e. following biliary tract cancer, RRs were increased for colorectal cancer (1.68, 1.05-2.54) and ovarian cancer (4.43, 2.29-7.74). Another study demonstrated an increased risk to develop gall bladder cancer in survivors of testicular germ cell tumors[2] and the same appears to be true for breast cancer[5]. An increased risk of biliary tract cancer at sites other than gall bladder or intrahepatic ducts has been observed in individuals who previously had been diagnosed with a Merkel cell carcinoma (SIR 7.24, 95% CI 1.46-21.16)[3]. Pancreatic cancer risk appears to be increased in patients previously diagnosed with gall bladder cancer[4].

General aspects to consider with respect to multiple primary tumors:
- Shared genetic (immune response, metabolic/hormonal/DNA-repair pathways) or non-genetic (chemical carcinogens, radiation, viruses, life-style) risk factors
- Therapy (radiation, chemotherapy, hormonal,...) related effects
- Possible bias because of increased surveillance and autopsy findings


[1] Su Y, Ahsan H, Neugut AI. The association between biliary tract cancers and cancers of other sites. Am J Gastroenterol 1999; 94(8):2256-2262.
[2] Richiardi L, Scélo G, Boffetta P, Hemminki K, Pukkala E, Olsen JH, Weiderpass E, Tracey E, Brewster DH, McBride ML, Kliewer EV, Tonita JM, Pompe-Kirn V, Kee-Seng C, Jonasson JG, Martos C, Brennan P. Second malignancies among survivors of germ-cell testicular cancer: a pooled analysis between 13 cancer registries. International journal of cancer 2007; 120(3):623-31.
[3] Howard RA, Dores GM, Curtis RE, Anderson WF, Travis LB. Merkel cell carcinoma and multiple primary cancers. Cancer epidemiology, biomarkers & prevention 2006; 15(8):1545-9.
[4] Shen M, Boffetta P, Olsen JH, Andersen A, Hemminki K, Pukkala E, Tracey E, Brewster DH, McBride ML, Pompe-Kirn V, Kliewer EV, Tonita JM, Chia KS, Martos C, Jonasson JG, Colin D, Scélo G, Brennan P. A pooled analysis of second primary pancreatic cancer. American journal of epidemiology 2006; 163(6):502-11.
[5] Levi F, Te VC, Randimbison L, La Vecchia C. Cancer risk in women with previous breast cancer. Annals of oncology : official journal of the European Society for Medical Oncology / ESMO 2003; 14(1):71-3.