Reduced Cancer Incidence in Huntington's Disease

Analysis in the Registry Study

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Pages (from-to)209-222
Number of pages14
JournalJournal of Huntington's Disease
Volume7
Issue number3
DOIs
Publication statusPublished - 2018

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Huntington Disease
Registries
Incidence
Neoplasms
Age of Onset
Population
Colorectal Neoplasms
Prostatic Neoplasms
Databases

Keywords

  • cancer
  • Huntington's disease
  • neurodegeneration
  • trinucleotide repeat

Cite this

@article{08a1d7676c4543589886ef0e14c2fc3f,
title = "Reduced Cancer Incidence in Huntington's Disease: Analysis in the Registry Study",
abstract = "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.",
keywords = "cancer, Huntington's disease, neurodegeneration, trinucleotide repeat",
author = "Paul McNulty and Richard Pilcher and Raviram Ramesh and Renata Necuiniate and Alis Hughes and Daniel Farewell and Peter Holmans and Lesley Jones and Bonelli, {Raphael M.} and Karen Hecht and Brigitte Herranhof and Anna Holl and Kapfhammer, {Hans Peter} and Michael Koppitz and Sabine Lilek and Markus Magnet and Nicole M{\"u}ller and Daniela Otti and Annamaria Painold and Karin Reisinger and Monika Scheibl and Helmut Sch{\"o}ggl and Jasmin Ullah and Braunwarth, {Eva Maria} and Florian Brugger and Lisa Buratti and Hametner, {Eva Maria} and Caroline Hepperger and Christiane Holas and Anna Hotter and Anna Hussl and Barbara Larcher and Philipp Mahlknecht and Christoph M{\"u}ller and Bernadette Pinter and Werner Poewe and Reiter, {Eva Magdalena} and Klaus Seppi and Fabienne Sprenger and Gregor Wenning and Gunther Ladurner and Stefan Lilek and Daniela Sinadinosa and Wolfgang Staffen and Walleczek, {Anna Maria} and Eric Constant and David Goudie and Taylor, {Anne Marie} and Gareth Thomas and Helen Murphy",
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language = "English",
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Reduced Cancer Incidence in Huntington's Disease : Analysis in the Registry Study. /.

In: Journal of Huntington's Disease, Vol. 7, No. 3, 2018, p. 209-222.

Research output: Contribution to journalArticle

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T1 - Reduced Cancer Incidence in Huntington's Disease

T2 - 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 - 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 - Constant, Eric

AU - Goudie, David

AU - Taylor, Anne Marie

AU - Thomas, Gareth

AU - Murphy, Helen

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

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