Reduced Cancer Incidence in Huntington's Disease: Analysis in the Registry Study

REGISTRY investigators of the European Huntington’s Disease Network

doi:10.3233/JHD-170263

Reduced Cancer Incidence in Huntington's Disease: Analysis in the Registry Study

REGISTRY investigators of the European Huntington’s Disease Network

Cardiff University
University of Chester
Medical University of Graz
Paracelsus Private Medical University
Friedrich-Alexander University Erlangen-Nürnberg
Innsbruck Medical University
Université catholique de Louvain
Institut de Pathologie et de Génétique (IPG)
European Huntington's Disease Network (EHDN)
KU Leuven
Fak Nemocnice Olomouc
Charles University
Aarhus University
University of Copenhagen
University of Southern Denmark
Ålands Hälso-och Sjukvård
Uppsala University
Department of Medical Genetics
University of Eastern Finland
Dep. of Neurology
Dep. of Medicak Genetics
Terveystalo Healthcare
Rehabilitation Centre Suvituuli
Université d'Angers
Groupe hospitalier Pellegrin
Hôpital Gabriel Montpied
Hôpital Henri Mondor
CHRU Roger Salengro
CHUNord
Assistance publique - Hôpitaux de Marseille
Sorbonne Université
University of Würzburg
Hôpital Charles Nicolle
Hôpital civil
Hôpital Purpan
RWTH Aachen University
University of Münster
Charité – Universitätsmedizin Berlin
Ruhr University Bochum
Reha Zentrum in Dinslaken im Gesundheitszentrums Lang
Technische Universität Dresden
University of Freiburg
University of Hamburg
Ulm University
Hannover Medical School
Schwerpunktpraxis Huntington
University of Marburg
Technical University of Munich
Isar-Amper-Klinikum - Klinik Taufkirchen (Vils)
University of Bari
University of Bologna
University of Florence
University of Genoa
IRCCS Fondazione Istituto Neurologico Carlo Besta - Milano
University of Naples Federico II
IRCCS Istituto Neurologico Mediterraneo Neuromed - Pozzilli (IS)
IRCCS Ospedale Casa Sollievo della Sofferenza - San Giovanni Rotondo (FG)
LIRH Foundation
Catholic University of the Sacred Heart
National Research Council of Italy
Sant'Andrea Hospital
Medisch Spectrum Twente
Polikliniek Neurologie
Radboud University Nijmegen
Leiden University
Maastricht University
University of Bergen
University of Oslo
Norwegian University of Science and Technology
Medical University of Gdańsk
Medical University of Silesia in Katowice
Krakowska Akademia Neurologii
University of Medical Sciences Poznan
Medical University of Warsaw
Institute of Psychiatry and Neurology, Warszawa
University of Coimbra
Hospital dos Capuchos
Centro Hospitalar Universitário Lisboa Norte
José de Mello Health
Centro Hospitalar Universitário de São João
Hospital Universitario Infanta Cristina
Hospital Universitari de Bellvitge
IDIBAPS
Barcelona-Hospital Mútua de Terrassa
Hospital Mare de Deu de La Merced
Hospital de La Santa Creu I Sant Pau
Hospital de Cruces
Hospital Universitario de Burgos
Maternity and Children's University Hospital
Hospital Universitario de Fuenlabrada
Hospital Universitario San Cecilio
Centro de Investigación de Enfermedades Neurológicas
Hospital Clínico San Carlos de Madrid
Hospital Ramon y Cajal
Fundación Jiménez Díaz
Hospital Universitario Son Espases
Hospital Virgen de la Arrixaca
Hospital Universitario Central de Asturias
Hospital Universitario de Navarra
Hospital Universitario Virgen del Rocio
Hospital Universitario Virgen Macarena
Residencia Santa Ana
Hospital Universitario La Fe
Sahlgrenska University Hospital
Karolinska Institutet
Umeå University
University of Bern
University of Lausanne
University of Zurich
University of Aberdeen
Royal Devon & Exeter NHS Foundation Trust
Northern Devon Healthcare NHS Trust
Birmingham and Solihull Mental Health NHS Foundation Trust
North Bristol NHS Trust
University of Cambridge
University of Manchester
Ninewells Hospital
NHS Lothian
NHS Fife
NHS Greater Glasgow and Clyde
Gloucestershire Hospitals NHS Foundation Trust
Hull University Teaching Hospitals NHS Trust
Leeds Teaching Hospitals NHS Trust
Millaton Court
Leicestershire Partnership Trust
The Walton Centre NHS Foundation Trust
University of London
University College London Hospitals NHS Foundation Trust
Guy's and St Thomas' NHS Foundation Trust
St Andrew's Healthcare
Lancashire Teaching Hospitals NHS Foundation Trust
Institute of Medical Genetics
Oxford University Hospitals NHS Foundation Trust
Mount Gould Hospital
University Hospitals Dorset NHS Foundation Trust
Sheffield Teaching Hospitals NHS Foundation Trust
University Hospital Southampton NHS Foundation Trust
Great Western Hospitals NHS Foundation Trust
University of Milan
CHDI Foundation, Inc.
Research Center of Neurology
2 mt Software Gmbh
University of Turku

Research output: Contribution to journal › Article › peer-review

29 Scopus citations

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 language	English
Pages (from-to)	209-222
Number of pages	14
Journal	Journal of Huntington's disease
Volume	7
Issue number	3
DOIs	https://doi.org/10.3233/JHD-170263
State	Published - 2018
Externally published	Yes

Bibliographical note

Publisher Copyright:
© 2018 - IOS Press and the authors. All rights reserved.

Funding

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). 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).

Funders	Funder number
CHDI Foundation	478
Medical Research Council	MR/P007015/1, MR/L02053X/1, MR/L010305/1, MR/L012936/1
Medical Research Council Canada

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

SDG 3 Good Health and Well-being

Keywords

Huntington's disease
cancer
neurodegeneration
trinucleotide repeat

Access to Document

10.3233/JHD-170263

Cite this

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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 = "Huntington's disease, cancer, neurodegeneration, trinucleotide repeat",

author = "\{REGISTRY investigators of the European Huntington{\textquoteright}s Disease Network\} and 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 Gillardin, \{Anne Fran{\c c}oise\} and L{\'e}onard, \{Marie Claude\} and Christine Verellen-Dumoulin and Sara Minster",

year = "2018",

doi = "10.3233/JHD-170263",

language = "אנגלית",

volume = "7",

pages = "209--222",

journal = "Journal of Huntington's disease",

issn = "1879-6397",

publisher = "SAGE Publications Ltd",

number = "3",

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T1 - Reduced Cancer Incidence in Huntington's Disease

T2 - Analysis in the Registry Study

AU - REGISTRY investigators of the European Huntington’s Disease Network

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 - Gillardin, Anne Françoise

AU - Léonard, Marie Claude

AU - Verellen-Dumoulin, Christine

AU - Minster, Sara

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.

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KW - cancer

KW - neurodegeneration

KW - trinucleotide repeat

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Reduced Cancer Incidence in Huntington's Disease: Analysis in the Registry Study

Abstract

Bibliographical note

Funding

UN SDGs

Keywords

Access to Document

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