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Decreased Cancer Risk for Patients With PolyQ Diseases

Cancer risk down in Huntington's disease, spinobulbar muscular atrophy, hereditary ataxia

THURSDAY, April 12 (HealthDay News) -- Patients with polyglutamine (polyQ) diseases have reduced incidence of cancer, according to a study published online April 12 in The Lancet Oncology.

To investigate the association of polyQ diseases with the risk of cancer, Jianguang Ji, M.D., from Lund University in Sweden, and colleagues analyzed data from 1,510 patients with Huntington's disease (HD), 471 with spinobulbar muscular atrophy (SBMA), and 3,425 with hereditary ataxia (HA), identified from 1969 to 2008 in Sweden. Data were linked to the Swedish Cancer Registry to calculate standardized incidence ratios for cancer and compare the risk with that of the general population.

The researchers found that cancer was diagnosed in 12.3 percent of patients with HA, 7.2 percent with SMBA, and 6 percent with HD; with standardized incidence ratios of 0.77, 0.65, and 0.47, respectively. The risk of cancer was even lower prior to diagnosis of polyQ disease. The risk of cancer and cancer incidence in unaffected parents of patients with polyQ were comparable to that seen in the general population.

"In this large study, we found that incidence of cancer, overall and for specific sites or types, was significantly lower in patients with polyQ diseases than in the general population," the authors write. "Future studies should investigate the specific biological mechanisms underlying the reduced cancer risk in patients with polyQ diseases."

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