在家族性腺多重症综合征中癌症发生率和死亡率
Dan Feldman1, Linda H Rodgers-Fouche2, Chinedu Ukaegbu3,4
1Division of Gastroenterology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts.
Diseases of the colon and rectum
|February 11, 2025
概括
结肠直肠癌仍然是家族性腺瘤多重症 (FAP) 的主要死亡原因,特别是在未被诊断的个体中. 结肠切除术后,胃和十二指肠癌成为FAP患者死亡的主要原因.
科学领域:
- 胃肠病学 胃肠病学
- 在瘤学瘤学.
- 遗传学 遗传学 是一个
背景情况:
- 家庭腺瘤多重症 (FAP) 管理包括降低风险的切除术.
- 在FAP患者中,结肠外恶性瘤在结肠切除术后的监测的重要性增加.
研究的目的:
- 确定目前所有癌症的发病率和家族性腺瘤多重症的死亡率.
- 在FAP中确定与癌症诊断和死亡率相关的风险因素.
主要方法:
- 追溯纵向队列研究.
- 参与了来自两个学术医院的358名患有APC致病变体或临床FAP标准的患者.
- 收集有关癌症诊断,死亡率和风险因素的数据.
主要成果:
- 大肠直肠癌 (CRC) 是最常见的恶性瘤 (16.5%),与新生FAP相关.
- 其他癌症包括甲状腺癌 (7.5%),十二指肠/小肠癌 (3.1%),胃癌 (2.8%).
- CRC导致10例死亡,所有这些都是未被诊断的经典FAP患者. 胃癌和十二指肠癌导致4例死亡,每例发生在切除肠道术后.
结论:
- 在FAP中,CRC仍然是主要的恶性瘤和死亡原因,特别是在未诊断的病例中.
- 在切除肠道术后的FAP患者中,胃癌和十二指肠/小肠癌现在是导致死亡的主要原因.
- 对结肠外恶性瘤的监测至关重要,特别是在切除术后.
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