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Incidence of and survival after subsequent cancers in carriers of pathogenic MMR variants with previous cancer: a report from the prospective Lynch syndrome database
- Source :
- Gut, Møller, P, Seppälä, T, Bernstein, I, Holinski-Feder, E, Sala, P, Evans, D G, Lindblom, A, Macrae, F, Blanco, I, Sijmons, R, Jeffries, J, Vasen, H, Burn, J, Nakken, S, Hovig, E, Rødland, E A, Tharmaratnam, K, de Vos Tot Nederveen Cappel, W H, Hill, J, Wijnen, J, Jenkins, M, Green, K, Lalloo, F, Sunde, L, Mints, M, Bertario, L, Pineda, M, Navarro, M, Morak, M, Renkonen-Sinisalo, L, Frayling, I M, Plazzer, J-P, Pylvanainen, K, Genuardi, M, Mecklin, J-P, Möslein, G, Sampson, J R, Capella, G & Mallorca Group (http://mallorca-group.org) 2017, ' Incidence of and survival after subsequent cancers in carriers of pathogenic MMR variants with previous cancer : a report from the prospective Lynch syndrome database ', Gut, vol. 66, no. 9, pp. 1657-1664 . https://doi.org/10.1136/gutjnl-2016-311403, Gut, 66(9), 1657-1664. BMJ PUBLISHING GROUP, Gut, 66(9), 1657-1664, Møller, P, Seppälä, T, Bernstein, I, Holinski-Feder, E, Sala, P, Evans, D G, Lindblom, A, Macrae, F, Blanco, I, Sijmons, R, Jeffries, J, Vasen, H, Burn, J, Nakken, S, Hovig, E, Rødland, E A, Tharmaratnam, K, de Vos Tot Nederveen Cappel, W H, Hill, J, Wijnen, J, Jenkins, M, Green, K, Lalloo, F, Sunde, L, Mints, M, Bertario, L, Pineda, M, Navarro, M, Morak, M, Renkonen-Sinisalo, L, Frayling, I M, Plazzer, J-P, Pylvanainen, K, Genuardi, M, Mecklin, J-P, Möslein, G, Sampson, J R, Capella, G & Mallorca Group (http://mallorca-group.org) 2016, ' Incidence of and survival after subsequent cancers in carriers of pathogenic MMR variants with previous cancer: a report from the prospective Lynch syndrome database. ', Gut . https://doi.org/10.1136/gutjnl-2016-311403, Dipòsit Digital de la UB, Universidad de Barcelona, Møller, P, Seppälä, T, Bernstein, I, Holinski-Feder, E, Sala, P, Evans, G, Lindblom, A, Macrae, F, Blanco, I, Sijmons, R, Jeffries, J, Vasen, H, Burn, J, Nakken, S, Hovig, E, Rødland, E A, Tharmaratnam, K, de Vos tot Nederveen Cappel, W H, Hill, J, Wijnen, J, Jenkins, M, Green, K, Lalloo, F, Sunde, L, Mints, M, Bertario, L, Pineda, M, Navarro, M, Morak, M, Renkonen-Sinisalo, L, Frayling, I M, Plazzer, J P, Pylvanainen, K, Genuardi, M, Mecklin, J P, Möslein, G, Sampson, J R & Capella, G 2016, ' Incidence of and survival after subsequent cancers in carriers of pathogenic MMR variants with previous cancer : a report from the prospective Lynch syndrome database ', Gut . https://doi.org/10.1136/gutjnl-2016-311403
- Publication Year :
- 2016
- Publisher :
- BMJ, 2016.
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Abstract
- OBJECTIVE: Today most patients with Lynch syndrome (LS) survive their first cancer. There is limited information on the incidences and outcome of subsequent cancers. The present study addresses three questions: (i) what is the cumulative incidence of a subsequent cancer; (ii) in which organs do subsequent cancers occur; and (iii) what is the survival following these cancers?DESIGN: Information was collated on prospectively organised surveillance and prospectively observed outcomes in patients with LS who had cancer prior to inclusion and analysed by age, gender and genetic variants.RESULTS: 1273 patients with LS from 10 countries were followed up for 7753 observation years. 318 patients (25.7%) developed 341 first subsequent cancers, including colorectal (n=147, 43%), upper GI, pancreas or bile duct (n=37, 11%) and urinary tract (n=32, 10%). The cumulative incidences for any subsequent cancer from age 40 to age 70 years were 73% for pathogenic MLH1 (path_MLH1), 76% for path_MSH2 carriers and 52% for path_MSH6 carriers, and for colorectal cancer (CRC) the cumulative incidences were 46%, 48% and 23%, respectively. Crude survival after any subsequent cancer was 82% (95% CI 76% to 87%) and 10-year crude survival after CRC was 91% (95% CI 83% to 95%).CONCLUSIONS: Relative incidence of subsequent cancer compared with incidence of first cancer was slightly but insignificantly higher than cancer incidence in patients with LS without previous cancer (range 0.94-1.49). The favourable survival after subsequent cancers validated continued follow-up to prevent death from cancer. The interactive website http://lscarisk.org was expanded to calculate the risks by gender, genetic variant and age for subsequent cancer for any patient with LS with previous cancer.
- Subjects :
- Male
Oncology
Survival
Colorectal cancer
Settore MED/03 - GENETICA MEDICA
GUIDELINES
DNA Mismatch Repair
0302 clinical medicine
Epidemiology
EPIDEMIOLOGY
Medicine
Cumulative incidence
Càncer
10. No inequality
Cancer
Factors de risc en les malalties
Incidence
Incidence (epidemiology)
Gastroenterology
Middle Aged
Lynch syndrome
3. Good health
DNA-Binding Proteins
Europe
MutS Homolog 2 Protein
030220 oncology & carcinogenesis
Colonic Neoplasms
Disease Progression
Female
030211 gastroenterology & hepatology
MutL Protein Homolog 1
Adult
medicine.medical_specialty
Risk factors in diseases
Colon
MLH1
Risk Assessment
RC0254
03 medical and health sciences
Internal medicine
MANAGEMENT
Humans
Supervivència
Germ-Line Mutation
Survival analysis
Aged
Neoplasm Staging
Gynecology
business.industry
COLORECTAL CANCER GENES
Genetic Variation
INHERITED CANCERS
medicine.disease
SCREENING
Colorectal Neoplasms, Hereditary Nonpolyposis
Survival Analysis
CANCER GENETICS
3121 General medicine, internal medicine and other clinical medicine
business
Subjects
Details
- ISSN :
- 14683288 and 00175749
- Volume :
- 66
- Database :
- OpenAIRE
- Journal :
- Gut
- Accession number :
- edsair.doi.dedup.....b9b8e48e999cf5d5c4793cfd0b05a7ca