BACKGROUND & AIMS: Lynch syndrome family members have a high risk of developing colorectal (CRC), endometrial (EC), and other cancers. A large-scale surveillance program was introduced in The Netherlands in the late 1980s. The aims of the study were to evaluate the effectiveness of this program by assessing mortality because of CRC and EC before and after 1990 and to compare mortality because of all cancers (except CRC/EC) with mortality in the general population. METHODS: Family members with at least 50% probability of being a carrier were selected for the study. The standardized mortality ratio (SMR) because of cancer and the absolute excess risk of death (AER) were calculated. RESULTS: In the total cohort (N = 2788), 445 subjects had died because of cancer. The 3 most frequent causes of cancer-related deaths were CRC (50.3%), EC (6.7%), and brain tumors (6.7%). A significant decrease (70%) in SMR for CRC over time was observed (P < .001); the SMR for EC showed no decreasing trend over time. A significantly increased SMR was found for cancer of the small bowel (SMR = 18.3), brain (SMR = 9.1), kidney/ureter (SMR = 5.9), ovarium (SMR = 2.3), pancreas (SMR = 2.2), and stomach (SMR = 2.1). The AER was significantly increased for brain tumors only. CONCLUSIONS: Since the introduction of surveillance, the mortality because of CRC has decreased. Except for brain tumors, we did not find a significantly increased AER for tumors other than CRC/EC.
BACKGROUND & AIMS:Lynch syndrome family members have a high risk of developing colorectal (CRC), endometrial (EC), and other cancers. A large-scale surveillance program was introduced in The Netherlands in the late 1980s. The aims of the study were to evaluate the effectiveness of this program by assessing mortality because of CRC and EC before and after 1990 and to compare mortality because of all cancers (except CRC/EC) with mortality in the general population. METHODS: Family members with at least 50% probability of being a carrier were selected for the study. The standardized mortality ratio (SMR) because of cancer and the absolute excess risk of death (AER) were calculated. RESULTS: In the total cohort (N = 2788), 445 subjects had died because of cancer. The 3 most frequent causes of cancer-related deaths were CRC (50.3%), EC (6.7%), and brain tumors (6.7%). A significant decrease (70%) in SMR for CRC over time was observed (P < .001); the SMR for EC showed no decreasing trend over time. A significantly increased SMR was found for cancer of the small bowel (SMR = 18.3), brain (SMR = 9.1), kidney/ureter (SMR = 5.9), ovarium (SMR = 2.3), pancreas (SMR = 2.2), and stomach (SMR = 2.1). The AER was significantly increased for brain tumors only. CONCLUSIONS: Since the introduction of surveillance, the mortality because of CRC has decreased. Except for brain tumors, we did not find a significantly increased AER for tumors other than CRC/EC.
Authors: María Dolores Giráldez; Francesc Balaguer; Luis Bujanda; Miriam Cuatrecasas; Jenifer Muñoz; Virginia Alonso-Espinaco; Mikel Larzabal; Anna Petit; Victoria Gonzalo; Teresa Ocaña; Leticia Moreira; José María Enríquez-Navascués; C Richard Boland; Ajay Goel; Antoni Castells; Sergi Castellví-Bel Journal: Clin Cancer Res Date: 2010-10-05 Impact factor: 12.531
Authors: Fay Kastrinos; Ewout W Steyerberg; Rowena Mercado; Judith Balmaña; Spring Holter; Steven Gallinger; Kimberly D Siegmund; James M Church; Mark A Jenkins; Noralane M Lindor; Stephen N Thibodeau; Lynn Anne Burbidge; Richard J Wenstrup; Sapna Syngal Journal: Gastroenterology Date: 2010-08-19 Impact factor: 22.682
Authors: S G Nicholls; B J Wilson; S M Craigie; H Etchegary; D Castle; J C Carroll; B K Potter; L Lemyre; J Little Journal: Genome Date: 2013-08-31 Impact factor: 2.166
Authors: Wouter H de Vos tot Nederveen Cappel; Heikki J Järvinen; Patrick M Lynch; Christoph Engel; Jukka-Pekka Mecklin; Hans F A Vasen Journal: Fam Cancer Date: 2013-06 Impact factor: 2.375