Incidence of and survival after subsequent cancers in carriers of pathogenic MMR variants with previous cancer: a report from the prospective Lynch syndrome database.
Incidence of and survival after subsequent cancers in carriers of pathogenic MMR variants with previous cancer: a report from the prospective Lynch syndrome database.
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DOI：
10.1136/gutjnl-2016-311403
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发表时间：
2017-09
期刊：
影响因子：
24.5
通讯作者：
Mallorca Group (http://mallorca-group.org)
中科院分区：
文献类型：
--
作者：
Møller P;Seppälä T;Bernstein I;Holinski-Feder E;Sala P;Evans DG;Lindblom A;Macrae F;Blanco I;Sijmons R;Jeffries J;Vasen H;Burn J;Nakken S;Hovig E;Rødland EA;Tharmaratnam K;de Vos Tot Nederveen Cappel WH;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 IM;Plazzer JP;Pylvanainen K;Genuardi M;Mecklin JP;Möslein G;Sampson JR;Capella G;Mallorca Group (http://mallorca-group.org)
今天，大多数林奇综合征（LS）患者在首次癌症中存活。有关后续癌症的发生率和结局的信息有限。本研究解决了三个问题：（i）后续癌症的累积发病率是多少;（ii）后续癌症发生在哪些器官中;以及（iii）这些癌症的生存率是多少？对入选前患有癌症的LS患者的前瞻性组织监测和前瞻性观察结局的信息进行了整理，并按年龄、性别和遗传变异进行了分析。对来自10个国家的1273例LS患者进行了7753个观察年的随访。318例患者（25.7%）发生了341例首次后续癌症，包括结直肠癌（n=147，43%）、上消化道、胰腺或胆管癌（n=37，11%）和尿路癌（n=32，10%）。从40岁到70岁，致病性MLH 1（path_MLH1）、path_MSH2携带者和path_MSH6携带者的任何后续癌症的累积发病率分别为73%、76%和52%，而结直肠癌（CRC）的累积发病率分别为46%、48%和23%。 任何后续癌症后的粗生存率为82%（95% CI 76%至87%），CRC后10年粗生存率为91%（95% CI 83%至95%）。与首次癌症发生率相比，后续癌症的相对发生率略高于无既往癌症的LS患者的癌症发生率（范围为0.94-1.49）。后续癌症后的良好生存证实了继续随访以预防癌症死亡。扩展了交互式网站http://lscarisk.org，以计算任何患有LS且既往患有癌症的患者的性别、遗传变异和年龄的后续癌症风险。
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? 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. 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%). 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.
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影响因子：
2.2
作者：
Talseth-Palmer, Bente A.;Wijnen, Juul T.;Grice, Desma M.;Scott, Rodney J.
通讯作者：
Scott, Rodney J.
影响因子：
24.5
作者：
Parry S;Win AK;Parry B;Macrae FA;Gurrin LC;Church JM;Baron JA;Giles GG;Leggett BA;Winship I;Lipton L;Young GP;Young JP;Lodge CJ;Southey MC;Newcomb PA;Le Marchand L;Haile RW;Lindor NM;Gallinger S;Hopper JL;Jenkins MA
通讯作者：
Jenkins MA
影响因子：
10.3
作者：
Win, Aung Ko;Lindor, Noralane M.;Jenkins, Mark A.
通讯作者：
Jenkins, Mark A.
影响因子：
2.2
作者：
Pylvanainen, Kirsi;Lehtinen, Tuula;Mecklin, Jukka-Pekka
通讯作者：
Mecklin, Jukka-Pekka
影响因子：
254.7
作者：
WARTHIN, AS
通讯作者：
WARTHIN, AS