Improved Survival Outcomes in Cancer Patients with Hereditary Hemorrhagic Telangiectasia
Improved Survival Outcomes in Cancer Patients with Hereditary Hemorrhagic Telangiectasia
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DOI：
10.1158/1055-9965.epi-13-0665
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发表时间：
2014-01-01
影响因子：
3.8
通讯作者：
Vary, Calvin P. H.
中科院分区：
文献类型：
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作者：
Duarte, Christine W.;Murray, Kimberly;Vary, Calvin P. H.
背景：遗传性出血性毛细血管扩张症（HHT）是一种以内皮糖蛋白（一种血管生成蛋白）缺乏为特征的遗传性疾病。内皮糖蛋白表达对动物研究中癌症结果的净效应已被证明是有争议的。我们评估了是否降低全身内皮素水平，预计在诊断为HHT的患者，影响cancer.Methods的临床结局：回顾性队列分析，使用监测，流行病学，和最终结果，医疗保险进行了评估的影响HHT对乳腺癌，结直肠癌，肺癌，前列腺癌患者的生存在2000年和2007年之间（n = 540，520）。我们生成了Kaplan-Meier生存曲线和考克斯模型，以比较HHT对四种癌症的全因生存率的影响，并按癌症分别建立模型，调整人口统计学变量、癌症类型、癌症分期和合并症。四种癌症的全因生存分析显示，调整后的HR为0.69 [95%置信区间（CI）为0.51-0.91; P = 0.009]，表明生存结局显著改善。当按癌症类型分层时，HHT诊断在乳腺癌患者中显示出显著的保护作用，校正HR为0.31（95% CI，0.13-0.75; P = 0.009）。HHT与乳腺癌、前列腺癌、结直肠癌和肺癌患者的生存结局改善之间存在显著相关性，在按癌症分层的分析中，影响：本研究支持系统性诱导内皮糖蛋白表达与多种癌症的生存结局改善相关的假设，并表明抗内皮糖蛋白抗体治疗可能具有广泛的应用。(C)AACR。
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 Surveillance, Epidemiology, and End Results-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-Meier 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 HR 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 HR of 0.31 (95% 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 educed endoglin expression is associated with improved survival outcome in multiple cancers, and suggests that anti-endoglin antibody therapy may have broad-based application. (C) AACR.