Cancer risk and survival in path_MMR carriers by gene and gender up to 75 years of age: a report from the Prospective Lynch Syndrome Database

Publication Date:
2018-06-08
Publisher:
BMJ Publishing Group
Print ISSN:
0017-5749
Electronic ISSN:
1468-3288
Topics:
Medicine
Keywords:
Clinical trials (epidemiology), Open access, Gut
Published by:
http://www.bmj.com/
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autor Moller, P., Seppälä, T. T., Bernstein, I., Holinski-Feder, E., Sala, P., Gareth Evans, D., Lindblom, A., Macrae, F., Blanco, I., Sijmons, R. H., Jeffries, J., Vasen, H. F. A., Burn, J., Nakken, S., Hovig, E., Rodland, E. A., Tharmaratnam, K., de Vos tot Nederveen Cappel, W. H., Hill, J., Wijnen, J. T., Jenkins, M. A., Green, K., Lalloo, F., Sunde, L., Mints, M., Bertario, L., Pineda, M., Navarro, M., Morak, M., Renkonen-Sinisalo, L., Valentin, M. D., Frayling, I. M., Plazzer, J.-P., Pylvanainen, K., Genuardi, M., Mecklin, J.-P., Moeslein, G., Sampson, J. R., Capella, G., in collaboration with The Mallorca Group
beschreibung Background Most patients with path_MMR gene variants (Lynch syndrome (LS)) now survive both their first and subsequent cancers, resulting in a growing number of older patients with LS for whom limited information exists with respect to cancer risk and survival. Objective and design This observational, international, multicentre study aimed to determine prospectively observed incidences of cancers and survival in path_MMR carriers up to 75 years of age. Results 3119 patients were followed for a total of 24 475 years. Cumulative incidences at 75 years (risks) for colorectal cancer were 46%, 43% and 15% in path_ MLH1 , path_ MSH2 and path_ MSH6 carriers; for endometrial cancer 43%, 57% and 46%; for ovarian cancer 10%, 17% and 13%; for upper gastrointestinal (gastric, duodenal, bile duct or pancreatic) cancers 21%, 10% and 7%; for urinary tract cancers 8%, 25% and 11%; for prostate cancer 17%, 32% and 18%; and for brain tumours 1%, 5% and 1%, respectively. Ovarian cancer occurred mainly premenopausally. By contrast, upper gastrointestinal, urinary tract and prostate cancers occurred predominantly at older ages. Overall 5-year survival for prostate cancer was 100%, urinary bladder 93%, ureter 85%, duodenum 67%, stomach 61%, bile duct 29%, brain 22% and pancreas 0%. Path_PMS2 carriers had lower risk for cancer. Conclusion Carriers of different path_MMR variants exhibit distinct patterns of cancer risk and survival as they age. Risk estimates for counselling and planning of surveillance and treatment should be tailored to each patient’s age, gender and path_MMR variant. We have updated our open-access website www.lscarisk.org to facilitate this.
citation_standardnr 6278850
datenlieferant ipn_articles
feed_id 3103
feed_publisher BMJ Publishing Group
feed_publisher_url http://www.bmj.com/
insertion_date 2018-06-08
journaleissn 1468-3288
journalissn 0017-5749
publikationsjahr_anzeige 2018
publikationsjahr_facette 2018
publikationsjahr_intervall 7984:2015-2019
publikationsjahr_sort 2018
publisher BMJ Publishing Group
quelle Gut
relation http://gut.bmj.com/cgi/content/short/67/7/1306?rss=1
schlagwort Clinical trials (epidemiology), Open access, Gut
search_space articles
shingle_author_1 Moller, P., Seppälä, T. T., Bernstein, I., Holinski-Feder, E., Sala, P., Gareth Evans, D., Lindblom, A., Macrae, F., Blanco, I., Sijmons, R. H., Jeffries, J., Vasen, H. F. A., Burn, J., Nakken, S., Hovig, E., Rodland, E. A., Tharmaratnam, K., de Vos tot Nederveen Cappel, W. H., Hill, J., Wijnen, J. T., Jenkins, M. A., Green, K., Lalloo, F., Sunde, L., Mints, M., Bertario, L., Pineda, M., Navarro, M., Morak, M., Renkonen-Sinisalo, L., Valentin, M. D., Frayling, I. M., Plazzer, J.-P., Pylvanainen, K., Genuardi, M., Mecklin, J.-P., Moeslein, G., Sampson, J. R., Capella, G., in collaboration with The Mallorca Group
shingle_author_2 Moller, P., Seppälä, T. T., Bernstein, I., Holinski-Feder, E., Sala, P., Gareth Evans, D., Lindblom, A., Macrae, F., Blanco, I., Sijmons, R. H., Jeffries, J., Vasen, H. F. A., Burn, J., Nakken, S., Hovig, E., Rodland, E. A., Tharmaratnam, K., de Vos tot Nederveen Cappel, W. H., Hill, J., Wijnen, J. T., Jenkins, M. A., Green, K., Lalloo, F., Sunde, L., Mints, M., Bertario, L., Pineda, M., Navarro, M., Morak, M., Renkonen-Sinisalo, L., Valentin, M. D., Frayling, I. M., Plazzer, J.-P., Pylvanainen, K., Genuardi, M., Mecklin, J.-P., Moeslein, G., Sampson, J. R., Capella, G., in collaboration with The Mallorca Group
shingle_author_3 Moller, P., Seppälä, T. T., Bernstein, I., Holinski-Feder, E., Sala, P., Gareth Evans, D., Lindblom, A., Macrae, F., Blanco, I., Sijmons, R. H., Jeffries, J., Vasen, H. F. A., Burn, J., Nakken, S., Hovig, E., Rodland, E. A., Tharmaratnam, K., de Vos tot Nederveen Cappel, W. H., Hill, J., Wijnen, J. T., Jenkins, M. A., Green, K., Lalloo, F., Sunde, L., Mints, M., Bertario, L., Pineda, M., Navarro, M., Morak, M., Renkonen-Sinisalo, L., Valentin, M. D., Frayling, I. M., Plazzer, J.-P., Pylvanainen, K., Genuardi, M., Mecklin, J.-P., Moeslein, G., Sampson, J. R., Capella, G., in collaboration with The Mallorca Group
shingle_author_4 Moller, P., Seppälä, T. T., Bernstein, I., Holinski-Feder, E., Sala, P., Gareth Evans, D., Lindblom, A., Macrae, F., Blanco, I., Sijmons, R. H., Jeffries, J., Vasen, H. F. A., Burn, J., Nakken, S., Hovig, E., Rodland, E. A., Tharmaratnam, K., de Vos tot Nederveen Cappel, W. H., Hill, J., Wijnen, J. T., Jenkins, M. A., Green, K., Lalloo, F., Sunde, L., Mints, M., Bertario, L., Pineda, M., Navarro, M., Morak, M., Renkonen-Sinisalo, L., Valentin, M. D., Frayling, I. M., Plazzer, J.-P., Pylvanainen, K., Genuardi, M., Mecklin, J.-P., Moeslein, G., Sampson, J. R., Capella, G., in collaboration with The Mallorca Group
shingle_catch_all_1 Cancer risk and survival in path_MMR carriers by gene and gender up to 75 years of age: a report from the Prospective Lynch Syndrome Database
Clinical trials (epidemiology), Open access, Gut
Background Most patients with path_MMR gene variants (Lynch syndrome (LS)) now survive both their first and subsequent cancers, resulting in a growing number of older patients with LS for whom limited information exists with respect to cancer risk and survival. Objective and design This observational, international, multicentre study aimed to determine prospectively observed incidences of cancers and survival in path_MMR carriers up to 75 years of age. Results 3119 patients were followed for a total of 24 475 years. Cumulative incidences at 75 years (risks) for colorectal cancer were 46%, 43% and 15% in path_ MLH1 , path_ MSH2 and path_ MSH6 carriers; for endometrial cancer 43%, 57% and 46%; for ovarian cancer 10%, 17% and 13%; for upper gastrointestinal (gastric, duodenal, bile duct or pancreatic) cancers 21%, 10% and 7%; for urinary tract cancers 8%, 25% and 11%; for prostate cancer 17%, 32% and 18%; and for brain tumours 1%, 5% and 1%, respectively. Ovarian cancer occurred mainly premenopausally. By contrast, upper gastrointestinal, urinary tract and prostate cancers occurred predominantly at older ages. Overall 5-year survival for prostate cancer was 100%, urinary bladder 93%, ureter 85%, duodenum 67%, stomach 61%, bile duct 29%, brain 22% and pancreas 0%. Path_PMS2 carriers had lower risk for cancer. Conclusion Carriers of different path_MMR variants exhibit distinct patterns of cancer risk and survival as they age. Risk estimates for counselling and planning of surveillance and treatment should be tailored to each patient’s age, gender and path_MMR variant. We have updated our open-access website www.lscarisk.org to facilitate this.
Moller, P., Seppälä, T. T., Bernstein, I., Holinski-Feder, E., Sala, P., Gareth Evans, D., Lindblom, A., Macrae, F., Blanco, I., Sijmons, R. H., Jeffries, J., Vasen, H. F. A., Burn, J., Nakken, S., Hovig, E., Rodland, E. A., Tharmaratnam, K., de Vos tot Nederveen Cappel, W. H., Hill, J., Wijnen, J. T., Jenkins, M. A., Green, K., Lalloo, F., Sunde, L., Mints, M., Bertario, L., Pineda, M., Navarro, M., Morak, M., Renkonen-Sinisalo, L., Valentin, M. D., Frayling, I. M., Plazzer, J.-P., Pylvanainen, K., Genuardi, M., Mecklin, J.-P., Moeslein, G., Sampson, J. R., Capella, G., in collaboration with The Mallorca Group
BMJ Publishing Group
0017-5749
00175749
1468-3288
14683288
shingle_catch_all_2 Cancer risk and survival in path_MMR carriers by gene and gender up to 75 years of age: a report from the Prospective Lynch Syndrome Database
Clinical trials (epidemiology), Open access, Gut
Background Most patients with path_MMR gene variants (Lynch syndrome (LS)) now survive both their first and subsequent cancers, resulting in a growing number of older patients with LS for whom limited information exists with respect to cancer risk and survival. Objective and design This observational, international, multicentre study aimed to determine prospectively observed incidences of cancers and survival in path_MMR carriers up to 75 years of age. Results 3119 patients were followed for a total of 24 475 years. Cumulative incidences at 75 years (risks) for colorectal cancer were 46%, 43% and 15% in path_ MLH1 , path_ MSH2 and path_ MSH6 carriers; for endometrial cancer 43%, 57% and 46%; for ovarian cancer 10%, 17% and 13%; for upper gastrointestinal (gastric, duodenal, bile duct or pancreatic) cancers 21%, 10% and 7%; for urinary tract cancers 8%, 25% and 11%; for prostate cancer 17%, 32% and 18%; and for brain tumours 1%, 5% and 1%, respectively. Ovarian cancer occurred mainly premenopausally. By contrast, upper gastrointestinal, urinary tract and prostate cancers occurred predominantly at older ages. Overall 5-year survival for prostate cancer was 100%, urinary bladder 93%, ureter 85%, duodenum 67%, stomach 61%, bile duct 29%, brain 22% and pancreas 0%. Path_PMS2 carriers had lower risk for cancer. Conclusion Carriers of different path_MMR variants exhibit distinct patterns of cancer risk and survival as they age. Risk estimates for counselling and planning of surveillance and treatment should be tailored to each patient’s age, gender and path_MMR variant. We have updated our open-access website www.lscarisk.org to facilitate this.
Moller, P., Seppälä, T. T., Bernstein, I., Holinski-Feder, E., Sala, P., Gareth Evans, D., Lindblom, A., Macrae, F., Blanco, I., Sijmons, R. H., Jeffries, J., Vasen, H. F. A., Burn, J., Nakken, S., Hovig, E., Rodland, E. A., Tharmaratnam, K., de Vos tot Nederveen Cappel, W. H., Hill, J., Wijnen, J. T., Jenkins, M. A., Green, K., Lalloo, F., Sunde, L., Mints, M., Bertario, L., Pineda, M., Navarro, M., Morak, M., Renkonen-Sinisalo, L., Valentin, M. D., Frayling, I. M., Plazzer, J.-P., Pylvanainen, K., Genuardi, M., Mecklin, J.-P., Moeslein, G., Sampson, J. R., Capella, G., in collaboration with The Mallorca Group
BMJ Publishing Group
0017-5749
00175749
1468-3288
14683288
shingle_catch_all_3 Cancer risk and survival in path_MMR carriers by gene and gender up to 75 years of age: a report from the Prospective Lynch Syndrome Database
Clinical trials (epidemiology), Open access, Gut
Background Most patients with path_MMR gene variants (Lynch syndrome (LS)) now survive both their first and subsequent cancers, resulting in a growing number of older patients with LS for whom limited information exists with respect to cancer risk and survival. Objective and design This observational, international, multicentre study aimed to determine prospectively observed incidences of cancers and survival in path_MMR carriers up to 75 years of age. Results 3119 patients were followed for a total of 24 475 years. Cumulative incidences at 75 years (risks) for colorectal cancer were 46%, 43% and 15% in path_ MLH1 , path_ MSH2 and path_ MSH6 carriers; for endometrial cancer 43%, 57% and 46%; for ovarian cancer 10%, 17% and 13%; for upper gastrointestinal (gastric, duodenal, bile duct or pancreatic) cancers 21%, 10% and 7%; for urinary tract cancers 8%, 25% and 11%; for prostate cancer 17%, 32% and 18%; and for brain tumours 1%, 5% and 1%, respectively. Ovarian cancer occurred mainly premenopausally. By contrast, upper gastrointestinal, urinary tract and prostate cancers occurred predominantly at older ages. Overall 5-year survival for prostate cancer was 100%, urinary bladder 93%, ureter 85%, duodenum 67%, stomach 61%, bile duct 29%, brain 22% and pancreas 0%. Path_PMS2 carriers had lower risk for cancer. Conclusion Carriers of different path_MMR variants exhibit distinct patterns of cancer risk and survival as they age. Risk estimates for counselling and planning of surveillance and treatment should be tailored to each patient’s age, gender and path_MMR variant. We have updated our open-access website www.lscarisk.org to facilitate this.
Moller, P., Seppälä, T. T., Bernstein, I., Holinski-Feder, E., Sala, P., Gareth Evans, D., Lindblom, A., Macrae, F., Blanco, I., Sijmons, R. H., Jeffries, J., Vasen, H. F. A., Burn, J., Nakken, S., Hovig, E., Rodland, E. A., Tharmaratnam, K., de Vos tot Nederveen Cappel, W. H., Hill, J., Wijnen, J. T., Jenkins, M. A., Green, K., Lalloo, F., Sunde, L., Mints, M., Bertario, L., Pineda, M., Navarro, M., Morak, M., Renkonen-Sinisalo, L., Valentin, M. D., Frayling, I. M., Plazzer, J.-P., Pylvanainen, K., Genuardi, M., Mecklin, J.-P., Moeslein, G., Sampson, J. R., Capella, G., in collaboration with The Mallorca Group
BMJ Publishing Group
0017-5749
00175749
1468-3288
14683288
shingle_catch_all_4 Cancer risk and survival in path_MMR carriers by gene and gender up to 75 years of age: a report from the Prospective Lynch Syndrome Database
Clinical trials (epidemiology), Open access, Gut
Background Most patients with path_MMR gene variants (Lynch syndrome (LS)) now survive both their first and subsequent cancers, resulting in a growing number of older patients with LS for whom limited information exists with respect to cancer risk and survival. Objective and design This observational, international, multicentre study aimed to determine prospectively observed incidences of cancers and survival in path_MMR carriers up to 75 years of age. Results 3119 patients were followed for a total of 24 475 years. Cumulative incidences at 75 years (risks) for colorectal cancer were 46%, 43% and 15% in path_ MLH1 , path_ MSH2 and path_ MSH6 carriers; for endometrial cancer 43%, 57% and 46%; for ovarian cancer 10%, 17% and 13%; for upper gastrointestinal (gastric, duodenal, bile duct or pancreatic) cancers 21%, 10% and 7%; for urinary tract cancers 8%, 25% and 11%; for prostate cancer 17%, 32% and 18%; and for brain tumours 1%, 5% and 1%, respectively. Ovarian cancer occurred mainly premenopausally. By contrast, upper gastrointestinal, urinary tract and prostate cancers occurred predominantly at older ages. Overall 5-year survival for prostate cancer was 100%, urinary bladder 93%, ureter 85%, duodenum 67%, stomach 61%, bile duct 29%, brain 22% and pancreas 0%. Path_PMS2 carriers had lower risk for cancer. Conclusion Carriers of different path_MMR variants exhibit distinct patterns of cancer risk and survival as they age. Risk estimates for counselling and planning of surveillance and treatment should be tailored to each patient’s age, gender and path_MMR variant. We have updated our open-access website www.lscarisk.org to facilitate this.
Moller, P., Seppälä, T. T., Bernstein, I., Holinski-Feder, E., Sala, P., Gareth Evans, D., Lindblom, A., Macrae, F., Blanco, I., Sijmons, R. H., Jeffries, J., Vasen, H. F. A., Burn, J., Nakken, S., Hovig, E., Rodland, E. A., Tharmaratnam, K., de Vos tot Nederveen Cappel, W. H., Hill, J., Wijnen, J. T., Jenkins, M. A., Green, K., Lalloo, F., Sunde, L., Mints, M., Bertario, L., Pineda, M., Navarro, M., Morak, M., Renkonen-Sinisalo, L., Valentin, M. D., Frayling, I. M., Plazzer, J.-P., Pylvanainen, K., Genuardi, M., Mecklin, J.-P., Moeslein, G., Sampson, J. R., Capella, G., in collaboration with The Mallorca Group
BMJ Publishing Group
0017-5749
00175749
1468-3288
14683288
shingle_title_1 Cancer risk and survival in path_MMR carriers by gene and gender up to 75 years of age: a report from the Prospective Lynch Syndrome Database
shingle_title_2 Cancer risk and survival in path_MMR carriers by gene and gender up to 75 years of age: a report from the Prospective Lynch Syndrome Database
shingle_title_3 Cancer risk and survival in path_MMR carriers by gene and gender up to 75 years of age: a report from the Prospective Lynch Syndrome Database
shingle_title_4 Cancer risk and survival in path_MMR carriers by gene and gender up to 75 years of age: a report from the Prospective Lynch Syndrome Database
timestamp 2025-06-30T23:35:26.683Z
titel Cancer risk and survival in path_MMR carriers by gene and gender up to 75 years of age: a report from the Prospective Lynch Syndrome Database
titel_suche Cancer risk and survival in path_MMR carriers by gene and gender up to 75 years of age: a report from the Prospective Lynch Syndrome Database
topic WW-YZ
uid ipn_articles_6278850