局部复发和转移性疾病在染细胞瘤和交感性偏角细胞瘤
Marta Araujo-Castro1,2, Iñigo García Sanz3, César Mínguez Ojeda4
1Endocrinology & Nutrition Department, Hospital Universitario Ramón y Cajal, Madrid, Spain.
Frontiers in endocrinology
|December 29, 2023
概括
乳色细胞瘤和交感性偏角细胞瘤 (PPGLs) 的复发与SDHB基因突变,较大的瘤和较高的诺美甲氨酸水平有关. 密切监测对于所有PPGL患者至关重要,特别是那些风险较高的患者.
科学领域:
- 内分泌学 在内分泌学.
- 在瘤学瘤学.
- 遗传学 是一个遗传学.
背景情况:
- 乳色细胞瘤和交感性偏角细胞瘤 (PPGLs) 是罕见的神经内分泌瘤.
- 了解复发模式对于患者的管理和预后至关重要.
研究的目的:
- 为了确定PPGL的复发率.
- 确定PPGL患者局部复发和/或转移性疾病的预测因素.
主要方法:
- 追溯多中心研究涉及303名来自19家西班牙医院的PPGL患者.
- 术术切除后的复发被定义为局部或转移性疾病.
- 统计分析以确定复发的独立预测因素.
主要成果:
- 总共分析了303名患者 (288名表色细胞瘤,15名PGL).
- 在4.8年的中位数随访后,7.9%的患者 (24/303) 观察到疾病复发.
- 复发的预测因素包括致病性SDHB基因变异 (HR 13.3),尿中较高的诺美甲尼水平 (HR 1.02),以及较大的瘤大小 (HR 1.01).
结论:
- PPGL复发与SDHB突变,较大的瘤大小和尿中诺米甲氨酸水平升高有关.
- 复发可以随时发生,需要严格的长期随访.
- 患有已确定的风险因素的患者需要加强监测,以便早期检测复发性疾病.
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