Reduced Cancer Incidence in Huntington's Disease: Analysis in the Registry Study
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Reduced Cancer Incidence in Huntington's Disease: Analysis in the Registry Study. / McNulty, Paul; Pilcher, Richard; Ramesh, Raviram; Necuiniate, Renata; Hughes, Alis; Farewell, Daniel; Holmans, Peter; Jones, Lesley; REGISTRY Investigators of the European Huntington’s Disease Network.
in: J HUNTINGTONS DIS, Jahrgang 7, Nr. 3, 2018, S. 209-222.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Reduced Cancer Incidence in Huntington's Disease: Analysis in the Registry Study
AU - McNulty, Paul
AU - Pilcher, Richard
AU - Ramesh, Raviram
AU - Necuiniate, Renata
AU - Hughes, Alis
AU - Farewell, Daniel
AU - Holmans, Peter
AU - Jones, Lesley
AU - REGISTRY Investigators of the European Huntington’s Disease Network
AU - Boelmans, Kai
AU - Ganos, Christos
AU - Goerendt, Ines Katharina
AU - Hidding, Ute
AU - Lewerenz, Jan
AU - Münchau, Alexander
AU - Orth, Michael
AU - Zittel, Simone
PY - 2018
Y1 - 2018
N2 - BACKGROUND: People with Huntington's disease (HD) have been observed to have lower rates of cancers.OBJECTIVE: To investigate the relationship between age of onset of HD, CAG repeat length, and cancer diagnosis.METHODS: Data were obtained from the European Huntington's disease network REGISTRY study for 6540 subjects. Population cancer incidence was ascertained from the GLOBOCAN database to obtain standardised incidence ratios of cancers in the REGISTRY subjects.RESULTS: 173/6528 HD REGISTRY subjects had had a cancer diagnosis. The age-standardised incidence rate of all cancers in the REGISTRY HD population was 0.26 (CI 0.22-0.30). Individual cancers showed a lower age-standardised incidence rate compared with the control population with prostate and colorectal cancers showing the lowest rates. There was no effect of CAG length on the likelihood of cancer, but a cancer diagnosis within the last year was associated with a greatly increased rate of HD onset (Hazard Ratio 18.94, p < 0.001).CONCLUSIONS: Cancer is less common than expected in the HD population, confirming previous reports. However, this does not appear to be related to CAG length in HTT. A recent diagnosis of cancer increases the risk of HD onset at any age, likely due to increased investigation following a cancer diagnosis.
AB - BACKGROUND: People with Huntington's disease (HD) have been observed to have lower rates of cancers.OBJECTIVE: To investigate the relationship between age of onset of HD, CAG repeat length, and cancer diagnosis.METHODS: Data were obtained from the European Huntington's disease network REGISTRY study for 6540 subjects. Population cancer incidence was ascertained from the GLOBOCAN database to obtain standardised incidence ratios of cancers in the REGISTRY subjects.RESULTS: 173/6528 HD REGISTRY subjects had had a cancer diagnosis. The age-standardised incidence rate of all cancers in the REGISTRY HD population was 0.26 (CI 0.22-0.30). Individual cancers showed a lower age-standardised incidence rate compared with the control population with prostate and colorectal cancers showing the lowest rates. There was no effect of CAG length on the likelihood of cancer, but a cancer diagnosis within the last year was associated with a greatly increased rate of HD onset (Hazard Ratio 18.94, p < 0.001).CONCLUSIONS: Cancer is less common than expected in the HD population, confirming previous reports. However, this does not appear to be related to CAG length in HTT. A recent diagnosis of cancer increases the risk of HD onset at any age, likely due to increased investigation following a cancer diagnosis.
KW - Journal Article
U2 - 10.3233/JHD-170263
DO - 10.3233/JHD-170263
M3 - SCORING: Journal article
C2 - 30103338
VL - 7
SP - 209
EP - 222
JO - J HUNTINGTONS DIS
JF - J HUNTINGTONS DIS
SN - 1879-6397
IS - 3
ER -
