TY - JOUR
T1 - Reduced Cancer Incidence in Huntington's Disease
T2 - Analysis in the Registry Study
AU - McNulty, Paul
AU - Ramesh, Raviram
AU - Necuiniate, Renata
AU - Hughes, Alis
AU - Farewell, Daniel
AU - Holmans, Peter
AU - Jones, Lesley
AU - Bonelli, Raphael M.
AU - Hecht, Karen
AU - Herranhof, Brigitte
AU - Holl, Anna
AU - Kapfhammer, Hans Peter
AU - Koppitz, Michael
AU - Lilek, Sabine
AU - Magnet, Markus
AU - Müller, Nicole
AU - Otti, Daniela
AU - Painold, Annamaria
AU - Reisinger, Karin
AU - Scheibl, Monika
AU - Schöggl, Helmut
AU - Ullah, Jasmin
AU - Braunwarth, Eva Maria
AU - Brugger, Florian
AU - Buratti, Lisa
AU - Hametner, Eva Maria
AU - Hepperger, Caroline
AU - Holas, Christiane
AU - Hotter, Anna
AU - Hussl, Anna
AU - Larcher, Barbara
AU - Mahlknecht, Philipp
AU - Müller, Christoph
AU - Pinter, Bernadette
AU - Poewe, Werner
AU - Reiter, Eva Magdalena
AU - Seppi, Klaus
AU - Sprenger, Fabienne
AU - Wenning, Gregor
AU - Ladurner, Gunther
AU - Lilek, Stefan
AU - Sinadinosa, Daniela
AU - Staffen, Wolfgang
AU - Walleczek, Anna Maria
AU - Jack, Roisin
AU - Matheson, Kirsty
AU - Miedzybrodzka, Zosia
AU - Rae, Daniela
AU - Simpson, Sheila A.
AU - Summers, Fiona
AU - REGISTRY Investigators of the European Huntington's Disease Network
N1 - Funding Information:
We wish to acknowledge the time and effort of all participants in this study. We thank Daniel Nelmes for helpful advice regarding cancer diagnoses. Funding: the European Huntington's Disease Network is funded by the CHDI Foundation, Inc.: this project was EHDN project 478, and this work was supported by the MRC (MR/L010305/1).
previously available at https://doi.org/10.3233/JHD-170263
Publisher Copyright:
© 2018 - IOS Press and the authors. All rights reserved.
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 - cancer
KW - Huntington's disease
KW - neurodegeneration
KW - trinucleotide repeat
UR - http://www.scopus.com/inward/record.url?scp=85051366290&partnerID=8YFLogxK
M3 - Article
C2 - 30103338
AN - SCOPUS:85051366290
SN - 1879-6397
VL - 7
SP - 209
EP - 222
JO - Journal of Huntington's disease
JF - Journal of Huntington's disease
IS - 3
ER -
