In JAMA this week, researchers in the US and Sweden assess cancer risk among patients with myotonic muscular dystrophy, autosomal-dominant multisystem neuromuscular disorder. Previous case reports have suggested that patients with this type of dystrophy, which is characterized by unstable nucleotide repeat expansions, may be at increased risk for malignancies. For this study, the researchers analyzed data from 1,658 MMD patients, and found that 104 developed cancer following their MMD diagnosis. This, the team says, translated to an observed cancer rate of 73.4 per 10,000 person years in MMD patients, compared to an expected rate of about 36.9 per 10,000 person years in the general population. "Specifically, we observed significant excess risks of cancers of the endometrium, brain, ovary, and colon," the authors write.
Also in JAMA this week, researchers in the US present a new process from the American Cancer Society for the creation of "trustworthy" cancer screening guidelines. Often, guidelines written by different organizations, even when based on the same evidence and data, can differ and therefore create confusion, the authors write. The new ACS method for creating guidelines is meant to be more transparent, consistent, and rigorous, and aligns with the principles of the Institute of Medicine. They encourage the creation of "a single generalist group for writing the guidelines, commissioning independent systematic evidence reviews, and clearly articulating the benefits, limitations, and harms associated with a screening test," the team adds.