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 | |
Publication status | Published - 1 Jan 2018 |
Externally published | Yes |
Keywords
- cancer
- Huntington's disease
- neurodegeneration
- trinucleotide repeat
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In: Journal of Huntington's Disease, Vol. 7, No. 3, 01.01.2018, p. 209-222.
Research output: Contribution to journal › Article › Research › peer-review
TY - JOUR
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 - REGISTRY Investigators of the European Huntington's Disease Network
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 - Van De Wyngaerde, Françoise
AU - Dupuis, Michel
AU - Minet, Cécile
AU - Ribaï, Pascale
AU - Van Paemel, Dominique
AU - Boogaerts, Andrea
AU - Vandenberghe, Wim
AU - Van Reijen, Dimphna
AU - Kaiserova, Michaela
AU - Šenkárová, Zuzana
AU - Klempíř, Jiří
AU - Majerová, Veronika
AU - Roth, Jan
AU - Madsen, Louise Hasselstrøm
AU - Møller, Anette Torvin
AU - Hjermind, Lena
AU - Jacobsen, Oda
AU - Lindquist, Suzanne
AU - Nielsen, Jørgen
AU - Regeur, Lisbeth
AU - Stockholm, Jette
AU - Larsen, Ida Unmack
AU - Vangsted-Hansen, Christina
AU - Vinther-Jensen, Tua
AU - Lolk, Annette
AU - Lundsgaard, Marianne
AU - Wermuth, Lene
AU - Andersson, Christian
AU - Nyberg, Clara
AU - Sundblom, Jimmy
AU - Peippo, Maarit
AU - Sipponen, Marjett
AU - Hartikainen, Paivi
AU - Ollokainen, Mari
AU - Åman, Jaana
AU - Ignatius, Jaakko
AU - Kärppä, Mikko
AU - Mustonen, Aki
AU - Kajula, Outi
AU - Jääskalainen, Outi
AU - Moilanen, Jukka
AU - Santala, Maire
AU - Eklund, Pia
AU - Hiivola, Heli
AU - Hyppönen, Hannele
AU - Martikainen, Kirsti
AU - Tuuha, Katri
AU - Allain, Philippe
AU - Bonneau, Dominique
AU - Bost, Marie
AU - Gohier, Bénédicte
AU - Guérid, Marie Anne
AU - Olivier, Audrey
AU - Prouzet, Julie
AU - Prundean, Adriana
AU - Scherer-Gagou, Clarisse
AU - Verny, Christophe
AU - Babiloni, Blandine
AU - Debruxelles, Sabrina
AU - Duché, Charlotte
AU - Goizet, Cyril
AU - Jameau, Laetitia
AU - Lafoucrière, Danielle
AU - Spampinato, Umberto
AU - Couttier, Julien
AU - Debilly, Bérengère
AU - Delaigue, Christine
AU - Durif, Franck
AU - Legendre, Perrine
AU - Loiseau, Sylvie
AU - Ulla, Miguel
AU - Vidal, Tiphaine
AU - Bachoud-Lévi, Anne Catherine
AU - Badei, Farideh
AU - Boissé, Marie Françoise
AU - Boudali, Lotfi
AU - De Langavant, Laurent Cleret
AU - Lemoine, Laurie
AU - Morgado, Graca
AU - Youssov, Katia
AU - Annic, Agnès
AU - Barthélémy, Recka
AU - De Bruycker, Christelle
AU - Cabaret, Maryline
AU - Carette, Anne Sophie
AU - Carrière, Nicolas
AU - Decorte, Eric
AU - Defebvre, Luc
AU - Delliaux, Marie
AU - Delval, Arnaud
AU - Depelchin, Alizé
AU - Destee, Alain
AU - Dewulf-Pasz, Nelly
AU - Dondaine, Thibaut
AU - Dugauquier, Florence
AU - Dujardin, Kathy
AU - Hopes, Lucie
AU - Krystkowiak, Pierre
AU - Lemaire, Marie Hélène
AU - Manouvrier, Sylvie
AU - Mutez, Eugénie
AU - Peter, Mireille
AU - Plomhause, Lucie
AU - Sablonnière, Bernard
AU - Simonin, Clémence
AU - Tard, Céline
AU - Thibault-Tanchou, Stéphanie
AU - Vuillaume, Isabelle
AU - Bellonet, Marcellin
AU - Benoit, Alexandra
AU - Berrisoul, Hassan
AU - Blin, Stéphanie
AU - Courtin, Françoise
AU - Duru, Cécile
AU - Fasquel, Véronique
AU - Flament, Mélanie
AU - Godefroy, Olivier
AU - Mantaux, Béatrice
AU - Playe, Alicia
AU - Roussel, Martine
AU - Tir, Mélissa
AU - Schüler, Béatrice
AU - Wannepain, Sandrine
AU - Azulay, Jean Philippe
AU - Chabot, Christelle
AU - Delfini, Marie
AU - Eusebio, Alexandre
AU - Fluchere, Frédérique
AU - Grosjean, Hélène
AU - Mundler, Laura
AU - Nowak, Marielle
AU - Raseta, Rolland
AU - Benaich, Sandra
AU - Brice, Alexis
AU - Charles, Perrine
AU - Durr, Alexandra
AU - Ewenczyk, Claire
AU - Francisque, Hélène
AU - Jauffret, Céline
AU - Justo, Damian
AU - Kassar, Abdulrahman
AU - Klebe, Stephan
AU - Lesne, Fabien
AU - Milani, Paolo
AU - Monin, Marie Lorraine
AU - Roze, Emmanuel
AU - Tataru, Alina
AU - Tchikviladzé, Maya
AU - Bioux, Sandrine
AU - Bliaux, Evangeline
AU - Girard, Carole
AU - Guyant-Maréchal, Lucie
AU - Hannequin, DIdier
AU - Hannier, Véronique
AU - Jourdain, Séverine
AU - Maltête, David
AU - Pouliquen, Dorothée
AU - Lagha-Boukbiza, Ouhaid
AU - Longato, Nadine
AU - Marcel, Christophe
AU - Phillipps, Clélie
AU - Rudolf, Gabrielle
AU - Steinmetz, Giséle
AU - Tranchant, Christine
AU - Wagner, Caroline
AU - Zimmermann, Marie Agathe
AU - Blondeau, Leily
AU - Calvas, Fabienne
AU - Cheriet, Samia
AU - Delabaere, Helène
AU - Demonet, Jean François
AU - Pariente, Jérémie
AU - Pierre, Michèle
AU - Rolland, Sandrine
AU - Kosinski, Christoph Michael
AU - Milkereit, Eva
AU - Probst, Daniela
AU - Reetz, Kathrin
AU - Sass, Christian
AU - Schiefer, Johannes
AU - Schlangen, Christiane
AU - Werner, Cornelius J.
AU - Beuth, Markus
AU - Gelderblom, Harald
AU - Priller, Josef
AU - Prüß, Harald
AU - Spruth, Eike
AU - Thiel, Silvia
AU - Andrich, Jürgen
AU - Ellrichmann, Gisa
AU - Herrmann, Lennard
AU - Hoffmann, Rainer
AU - Kaminski, Barbara
AU - Kraus, Peter
AU - Saft, Carsten
AU - Stamm, Christiane
AU - Lange, Herwig
AU - Maiwald, Robert
AU - Bosredon, Cecile
AU - Hunger, Ulrike
AU - Ure, Alexandra
PY - 2018/1/1
Y1 - 2018/1/1
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
U2 - 10.3233/JHD-170263
DO - 10.3233/JHD-170263
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 -