A population-based survey of risk for cancer in individuals diagnosed with myotonic dystrophy.
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ABSTRACT: Introduction
The risk of cancer in patients diagnosed with myotonic dystrophy (DM) is reported for the homogeneous Utah population.Methods
Clinical data accessed from the largest Utah healthcare providers have been record-linked to the Utah Population Database, a population-based resource also linked to the Utah Cancer Registry. Relative risks were estimated for 36 cancers of different types in 281 DM patients.Results
Testicular cancer (relative risk [RR] = 10.74; 95% confidence interval [CI], 1.91-38.79), endometrial cancer (RR = 6.98; 95% CI, 1.24-25.22), and non-Hodgkin lymphoma (RR = 4.25; 95% CI, 1.16-12.43) were all observed at significant excess in DM patients.Conclusions
This study confirms an overall increased risk of cancer in DM. Individuals diagnosed with DM might benefit from risk counseling. Muscle Nerve 54: 783-785, 2016.
SUBMITTER: Abbott D
PROVIDER: S-EPMC5824717 | biostudies-literature | 2016 Oct
REPOSITORIES: biostudies-literature
Publications
A population-based survey of risk for cancer in individuals diagnosed with myotonic dystrophy.
Abbott Diana D Johnson Nicholas E NE Cannon-Albright Lisa A LA
Muscle & nerve 20160707 4
<h4>Introduction</h4>The risk of cancer in patients diagnosed with myotonic dystrophy (DM) is reported for the homogeneous Utah population.<h4>Methods</h4>Clinical data accessed from the largest Utah healthcare providers have been record-linked to the Utah Population Database, a population-based resource also linked to the Utah Cancer Registry. Relative risks were estimated for 36 cancers of different types in 281 DM patients.<h4>Results</h4>Testicular cancer (relative risk [RR] = 10.74; 95% con ...[more]
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Project description:Recent studies show that patients with myotonic dystrophy (DM) have an increased risk of specific malignancies, but estimates of absolute cancer risk accounting for competing events are lacking. Using the Swedish Patient Registry, we identified 1,081 patients with an inpatient and/or outpatient diagnosis of DM between 1987 and 2007. Date and cause of death and date of cancer diagnosis were extracted from the Swedish Cause of Death and Cancer Registries. We calculated non-parametric estimates of absolute cancer risk and cancer mortality accounting for the high non-cancer competing mortality associated with DM. Absolute cancer risk after DM diagnosis was 1.6% (95% CI=0.4-4%), 5% (95% CI=3-9%) and 9% (95% CI=6-13%) at ages 40, 50 and 60 years, respectively. Females had a higher absolute risk of all cancers combined than males: 9% (95% CI=4-14), and 13% (95% CI=9-20) vs. 2% (95%CI= 0.7-6) and 4% (95%CI=2-8) by ages 50 and 60 years, respectively) and developed cancer at younger ages (median age =51 years, range=22-74 vs. 57, range=43-84, respectively, p=0.02). Cancer deaths accounted for 10% of all deaths, with an absolute cancer mortality risk of 2% (95%CI=1-4.5%), 4% (95%CI=2-6%), and 6% (95%CI=4-9%) by ages 50, 60, and 70 years, respectively. No gender difference in cancer-specific mortality was observed (p=0.6). In conclusion, cancer significantly contributes to morbidity and mortality in DM patients, even after accounting for high competing DM mortality from non-neoplastic causes. It is important to apply population-appropriate, validated cancer screening strategies in DM patients.
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