荷兰家族腺多重症患者癌症发生率:全国性队列研究
Hicham Bouchiba1, Arthur S Aelvoet1, Mariëtte C A van Kouwen2
1Department of Gastroenterology and Hepatology, Amsterdam University Medical Center, Amsterdam, the Netherlands; Cancer Center Amsterdam, Amsterdam, the Netherlands; Amsterdam Gastroenterology Endocrinology Metabolism, Amsterdam, the Netherlands.
Gastroenterology
|February 25, 2026
概括
家庭腺瘤多重症 (FAP) 患者面临各种癌症的高风险,包括胃和十二指肠癌,尽管进行了预防性手术. 加强监测对于管理FAP个体这些增加的癌症风险至关重要.
科学领域:
- 遗传学和瘤学 在
- 流行病学 流行病学
- 遗传性癌症综合征 遗传性癌症综合征
背景情况:
- 家族性腺瘤多重症 (FAP) 显著增加了终身癌症风险.
- 虽然由于手术,结肠直肠癌 (CRC) 发病率有所下降,但其他癌症的风险有所增加.
- 了解FAP当前的累积癌症风险对于管理至关重要.
研究的目的:
- 评估荷兰FAP患者各种癌症的累积发病率和标准化风险.
- 评估近几十年来FAP中的癌症趋势.
- 为改善FAP的查和监测策略提供信息.
主要方法:
- 来自荷兰登记处的FAP患者的全国性队列研究 (1975-2024).
- 采用Fine和Gray竞争风险方法进行发病率估计.
- 与一般人群相比计算的标准化发病率 (SIRs).
主要成果:
- 确定了1230名FAP患者;388人发展了461种癌症.
- 对胃 (SIR 12.02),十二指肠 (SIR 277.28),囊 (SIR 113.85),CRC (SIR 14.22) 和小肠癌 (SIR 122.03) 观察到显著增加的风险.
- 此外,肝母细胞瘤 (SIR 747.52),甲状腺癌 (SIR 17.30),妇科癌 (SIR 2.27) 和中枢神经系统癌 (SIR 3.79) 的风险增加. 胃癌的发病率正在上升.
结论:
- 患有FAP的患者持续存在多种胃肠道和肠外癌症的高风险.
- 目前的预防措施需要补充,加强查和监测协议.
- 该研究强调了在FAP管理中持续需要保持警的监测.
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