系统治疗先进的乳红细胞瘤和帕拉格林瘤:来自单个中心队列的现实世界证据
Yun Liang1, Junyan Xu2, Dan Huang3
11Centre for Neuroendocrine Tumors, Fudan University Shanghai Cancer Center, Shanghai 200032, China.
Endocrine-related cancer
|January 16, 2026
概括
针对性治疗和体静止素类似物 (SSAs) 等全身疗法显示,对帕拉结瘤和体红细胞瘤 (PPGL) 患者的无进展生存期 (PFS) 比化疗更好,特别是在一线治疗中. 像SDHx这样的基因变异没有明显影响治疗反应.
科学领域:
- 在瘤学瘤学.
- 内分泌学 在内分泌学.
- 遗传学 遗传学 是一个
背景情况:
- 帕拉格林瘤和叶染色细胞瘤 (PPGLs) 是罕见的神经内分泌瘤,具有不同的遗传和临床特征.
- 关于PPGL系统治疗有效性的证据有限.
研究的目的:
- 评估针对性治疗,化疗和体静止素类似物 (SSA) 在PPGL患者的疗效.
- 根据治疗类型和遗传突变,分析无进展生存期 (PFS).
主要方法:
- 在2021年至2024年期间接受治疗的157名PPGL患者的回顾性审查.
- 收集临床,病理,遗传和治疗数据.
- 对针对性治疗,化疗和SSAs的PFS分析,包括surufatinib,temozolomide和lanreotide等特定药物.
主要成果:
- 113名患者的下一代测序在47.8%的患者中揭示了致病变体,SDHB是最常见的.
- 整体队列中PFS中位数:向治疗 (8.02个月),化疗 (5.06个月),SSAs (16.03个月).
- 与化疗相比,一线向治疗和SSAs显示出更优异的PFS (20.01和15.05个月与4.07个月,P <0.05).
结论:
- 第一线向治疗和SSAs为PPGLs提供了比化疗更长的PFS.
- 苏鲁法提尼布和兰列酸表明了有利的疾病控制.
- SDHx变体状态没有显示出与针对性治疗或temozolomide的反应有明确的关联,需要进一步验证.
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