Authors: Duarte CW, Murray K, Lucas FL, Fairfield K, Miller H, Brooks PC, Vary CP
Title: Improved Survival Outcomes in Cancer Patients with Hereditary Hemorrhagic Telangiectasia.
Journal: Cancer Epidemiol Biomarkers Prev :-
Date: 2013 Nov 05
Abstract: Background: Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder characterized by deficiency in endoglin, an angiogenic protein. The net effect of endoglin expression on cancer outcomes from animal studies has proven controversial. We evaluated whether reduced systemic endoglin levels, expected in patients diagnosed with HHT, impacted clinical outcomes for cancer. Methods: A retrospective cohort analysis using SEER-Medicare was conducted to evaluate the effect of HHT on survival among patients diagnosed with breast, colorectal, lung, or prostate cancer between 2000 and 2007 (n=540,520). We generated Kaplan-Meir survival curves and Cox models to compare the effect of HHT on all cause survival for a composite of the four cancers, and separate models by cancer, adjusting for demographic variables, cancer type, cancer stage, and comorbidities. Results: All cause survival analysis for a composite of the four cancers showed an adjusted hazard ratio of 0.69 (95% confidence interval (CI) of 0.51-0.91, p=0.009) for HHT, indicating significantly improved survival outcome. When stratified by cancer type, HHT diagnosis showed a significant protective effect among breast cancer patients with an adjusted hazard ratio of 0.31 (CI: 0.13-0.75, p=0.009). Conclusions: There was a significant association between HHT and improved survival outcome for a composite of patients with breast, prostate, colorectal, and lung cancer, and in analysis stratified by cancer, the association was significant for HHT patients with breast cancer. Impact: This study supports the hypothesis that systemically-reduced endoglin expression is associated with improved survival outcome in multiple cancers, and suggests that anti-endoglin antibody therapy may have broad-based application.
Last Modified: 03 Sep 2013