Should people with myotonic dystrophy be counselled on the risk of cancer?

A population-based survey of risk for cancer in individuals diagnosed with myotonic dystrophy

Abbott D, Johnson NE, Cannon-Albright LA

Muscle Nerve 2016; 54:783-785.



The risk of cancer in patients diagnosed with myotonic dystrophy (DM) is reported for the homogeneous Utah population.


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.


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.


This study confirms an overall increased risk of cancer in DM. Individuals diagnosed with DM might benefit from risk counseling.

This reference is cited in the neurochecklist:

Myotonic dystrophy type 1: management

Abstract link

By National Center for Biotechnology Information – NCBI Bookshelf “Genes and Disease” (1998-). Available from:, Public Domain, Link

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