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

• Published in: Journal of Huntington's disease 2018-01, Vol.7 (3), p.209
• Main Authors: Hepperger, Caroline, Klempíř, Jiří, Regeur, Lisbeth, Vinther-Jensen, Tua, Ollokainen, Mari, Kärppä, Mikko, Prouzet, Julie, Laurent Cleret de Langavant, Lemoine, Laurie, Carrière, Nicolas, Dugauquier, Florence, Mireille, Peter, Godefroy, Olivier, Durr, Alexandra, Francisque, Hélène, Jauffret, Céline, Kassar, Abdulrahman, Longato, Nadine, Phillipps, Clélie, Cheriet, Samia, Kosinski, Christoph Michael, Prüß, Harald, Saft, Carsten, Bosredon, Cecile, Maass, Antonia, Ossig, Christiana, Böringer, Regina, Heinicke, Walburgis, Lewerenz, Jan, Tacik, Pawel, Longinus, Bernhard, Niess, Anke, Franco, Giovanni, de Tommaso, Marina, Poda, Roberto, Bertini, Elisabetta, Nanetti, Lorenzo, Panzeri, Marta, Frontali, Marina, Modoni, Anna, Piccininni, Chiara, Ristori, Giovanni, Leenders, Nico, Tyvoll, Hilde, Bjørgo, Kathrine, Heiberg, Arvid, Dziadkiewicz, Artur, Slawek, Jaroslaw, Arkuszewski, Michał, Szczudlik, Andrzej, Ciesielska, Anna, Wiśniewski, Bartłomiej, Jachinska, Katarzyna, Sienkiewicz-Jarosz, Halina, Ziora-Jakutowicz, Karolina, Costa, Cristina, Andrade, Carlos, Carmen Durán Herrera, Ramon Fernandez de Bobadilla, Villa, Carolina, María del Valle Loarte, Maria Ascension Zea Sevilla, Moreau María Dolores Alarcón, Juan Marín Muñoz, Luis Menéndez Guisasola, Marta Para Prieto, Laura Vargas González, Romero Lemos, María Dolores, Carmen Peiró Vilaplana, Paloma Millan Palanca, Berglund, Maria, Westman, Laila, Wikström, Birgitta, Sabine Weber Zaugg, Mihaylova, Violeta, Matheson, Kirsty, Hill, Susan, McGill, Marie, Markova, Ivana, Peacy, Caroline, Yardumian, Pam, Freire-Patino, Dawn, Golding, Charlotte, Laing, Hana, Chu, Elvina, Hensman, Davina, Taylor, Rachel, Stopford, Cheryl, Morgan, Karen, Parkinson, Andrew, Soltysiak, Beverley, Williams, Josh, Fillingham, Kay, Gunner, Kerry, McLean, Tim, Come, Adrien, Finisterra, Ana Maria, Gilling, Mette, Münkel, Kristina, Marie-Noelle Witjes-Ané
• Format: Article
• Language: English
• Subjects: Age; Cancer; Diagnosis; Huntington's disease; Medical diagnosis; Polyglutamine; Population; Population studies; Prostate; Trinucleotide repeat diseases; Trinucleotide repeats
• ISSN: 1879-6397; 1879-6397
• DOI: 10.3233/JHD-170263

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