Literature DB >> 27638773

Cancer incidence in patients with hereditary hemorrhagic telangiectasia.

Christine W Duarte1, Adam W Black2, F Lee Lucas2, Calvin P H Vary3.   

Abstract

PURPOSE: Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder characterized by deficiency in endoglin, an angiogenic protein. We previously showed that HHT, in which systemic endoglin expression is reduced, was associated with better survival outcomes in cancer patients (Duarte et al. in Cancer Epidemiol Biomarkers Prev 23:117-125, 2014). Here, we evaluated whether HHT was associated with reduced cancer incidence.
METHODS: A matched case-control analysis using SEER Medicare was conducted to evaluate the effect of HHT on diagnosis with breast, colorectal, lung, or prostate cancer between 2000 and 2007 (n = 633,162). Cancer and non-cancer patients were matched on age, sex, SEER registry region, and length of the ascertainment period for HHT. We assessed crude association using a McNemar's test and then adjusted for demographic variables, cancer type, cancer stage, comorbidities, and ascertainment period with a conditional logistic regression model for cancer incidence.
RESULTS: The McNemar's test showed no significant association between HHT and cancer incidence (p = 0.74). Adjusting for covariates with the conditional logistic regression model did not change the result [HHT odds ratio 0.978; 95 % CI (0.795, 1.204)]. The lack of association between HHT and cancer incidence is unexpected given the previously discovered significant association between HHT and improved survival outcomes (Duarte et al. in Cancer Epidemiol Biomarkers Prev 23:117-125, 2014).
CONCLUSIONS: We conclude that the protective effect of reduced systemic endoglin expression in cancer is specific to cancer progression through its effect on vascularization and other stromal effects but does not extend to cancer initiation.

Entities:  

Keywords:  Cancer incidence; Case–control; Endoglin; HHT; SEER Medicare

Mesh:

Year:  2016        PMID: 27638773      PMCID: PMC5263109          DOI: 10.1007/s00432-016-2272-7

Source DB:  PubMed          Journal:  J Cancer Res Clin Oncol        ISSN: 0171-5216            Impact factor:   4.553


  15 in total

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5.  A phase I first-in-human study of TRC105 (Anti-Endoglin Antibody) in patients with advanced cancer.

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Review 7.  HHT: a rare disease with a broad spectrum of clinical aspects.

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9.  Endoglin, a TGF-beta binding protein of endothelial cells, is the gene for hereditary haemorrhagic telangiectasia type 1.

Authors:  K A McAllister; K M Grogg; D W Johnson; C J Gallione; M A Baldwin; C E Jackson; E A Helmbold; D S Markel; W C McKinnon; J Murrell
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10.  Endoglin-mediated suppression of prostate cancer invasion is regulated by activin and bone morphogenetic protein type II receptors.

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2.  20-year follow-up study of Danish HHT patients-survival and causes of death.

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3.  Altered hypoxia inducible factor regulation in hereditary haemorrhagic telangiectasia.

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Review 4.  Hot and Cold Tumors: Is Endoglin (CD105) a Potential Target for Vessel Normalization?

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