N Jewel Samadder1,2, Ken Robert Smith3,4, Jathine Wong3, Heidi Hanson3,5, Kenneth Boucher3,6, Randall W Burt3,7,8, Michael Charlton9, Kathryn R Byrne7, Juan F Gallegos-Orozco7, Cathryn Koptiuch3, Karen Curtin3,10. 1. Cancer Control and Population Sciences, Huntsman Cancer Institute, 2000 Circle of Hope, Salt Lake City, UT, 84112, USA. Jewel.samadder@hsc.utah.edu. 2. Department of Medicine (Gastroenterology), University of Utah, Salt Lake City, UT, USA. Jewel.samadder@hsc.utah.edu. 3. Cancer Control and Population Sciences, Huntsman Cancer Institute, 2000 Circle of Hope, Salt Lake City, UT, 84112, USA. 4. Department of Family and Consumer Studies, University of Utah, Salt Lake City, UT, USA. 5. Department of Family and Preventive Medicine, University of Utah, Salt Lake City, UT, USA. 6. Department of Medicine (Epidemiology), University of Utah, Salt Lake City, UT, USA. 7. Department of Medicine (Gastroenterology), University of Utah, Salt Lake City, UT, USA. 8. Department of Oncological Sciences, University of Utah, Salt Lake City, UT, USA. 9. Department of Medicine, Intermountain Healthcare, Salt Lake City, UT, USA. 10. Department of Medicine (Genetic Epidemiology), University of Utah, Salt Lake City, UT, USA.
Abstract
BACKGROUND AND OBJECTIVES: Biliary tract cancers (BTC) including, cholangiocarcinoma (CC) and gallbladder cancer (GBC), are rare and highly fatal malignancies. The etiology and inherited susceptibility of both malignancies are poorly understood. We quantified the risk of BTC in first-degree (FDR), second-degree (SDR), and first cousin (FC) relatives of individuals with BTC, stratified by tumor subsite. METHODS: BTC diagnosed between 1980 and 2011 were identified from the Utah Cancer Registry and linked to pedigrees from the Utah Population Database. Age- and gender-matched BTC-free controls were selected to form the comparison group for determining BTC risk in relatives using Cox regression analysis. RESULTS: Of the 1302 index patients diagnosed with BTC, 550 (42.2 %) were located in the gallbladder and 752 (57.8 %) were cholangiocarcinomas. There was no elevated risk of BTC (all subsites combined) in FDRs (HR 0.94, 95 % CI 0.29-3.0), SDRs (HR 0.25, 95 % CI 0.06-1.03), and FCs (HR 0.96, 95 % CI 0.61-1.51) of BTC cases compared to cancer-free controls. Similarly, no increased familial risk of GBC or CC was found in relatives of BTC patients stratified by tumor subsite compared to relatives of controls. CONCLUSIONS: Relatives of BTC patients are not at an increased risk of GBC or CC in a statewide population. This suggests that biliary tract cancer risk is not associated with a familial predisposition and may be mitigated more strongly by environmental modifiers.
BACKGROUND AND OBJECTIVES:Biliary tract cancers (BTC) including, cholangiocarcinoma (CC) and gallbladder cancer (GBC), are rare and highly fatal malignancies. The etiology and inherited susceptibility of both malignancies are poorly understood. We quantified the risk of BTC in first-degree (FDR), second-degree (SDR), and first cousin (FC) relatives of individuals with BTC, stratified by tumor subsite. METHODS: BTC diagnosed between 1980 and 2011 were identified from the Utah Cancer Registry and linked to pedigrees from the Utah Population Database. Age- and gender-matched BTC-free controls were selected to form the comparison group for determining BTC risk in relatives using Cox regression analysis. RESULTS: Of the 1302 index patients diagnosed with BTC, 550 (42.2 %) were located in the gallbladder and 752 (57.8 %) were cholangiocarcinomas. There was no elevated risk of BTC (all subsites combined) in FDRs (HR 0.94, 95 % CI 0.29-3.0), SDRs (HR 0.25, 95 % CI 0.06-1.03), and FCs (HR 0.96, 95 % CI 0.61-1.51) of BTC cases compared to cancer-free controls. Similarly, no increased familial risk of GBC or CC was found in relatives of BTC patients stratified by tumor subsite compared to relatives of controls. CONCLUSIONS: Relatives of BTC patients are not at an increased risk of GBC or CC in a statewide population. This suggests that biliary tract cancer risk is not associated with a familial predisposition and may be mitigated more strongly by environmental modifiers.
Entities:
Keywords:
Biliary tract; Cholangiocarcinoma; Familial; Gallbladder cancer
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