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[复杂上腺染细胞瘤和上腺瘤的转移风险预测和分级系统]

L L Wang¹, S M Yang², X J Wei¹

¹Department of Pathology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China.

Zhonghua bing li xue za zhi = Chinese journal of pathology

|March 8, 2026

概括

综合性肌肉细胞瘤和副瘤分级系统 (COPPS) 评分为3或更高,表明肌肉细胞瘤和副瘤 (PPGL) 转移或复发的风险更高. 将COPPS与SDHB和S-100蛋白表达结合起来,可以改善PPGL患者的风险分层.

科学领域:

内分泌学在内分泌学.
在瘤学瘤学.
病理学病理学病理学

背景情况:

乳红细胞瘤和偏角细胞瘤 (PPGL) 是一种罕见的神经内分泌瘤.
准确的分级系统对于预测PPGL转移和复发至关重要.

研究的目的:

为了评估PPGL的复合乳红细胞瘤和偏角细胞瘤分级系统 (COPPS).
在预测转移/复发方面,将COPPS与PASS和GAPP进行比较.
探索与PPGL转移/复发相关的基因突变.

主要方法:

对186个PPGL病例进行了回顾性分析.
对COPPS,PASS和GAPP预测值的评估.
SDHB和S-100蛋白质的免疫组织化学.
为了基因突变分析,进行全外体序列测序.

主要成果:

一个COPPS得分≥3与显著更高的转移/复发率 (54.6%对比<3组) 相关.
在预测转移/复发方面,COPPS表现出比GAPP更高的灵敏度 (88.3%),比PASS更高的特异性 (65.1%).
分别在50例和96例中观察到负的SDHB和S-100蛋白表达.
基因突变分析显示了SDHA,MDH2,MEN1,EGLN1,RET和SDHB中的病原性生殖基因突变,以及ATRX,KIF1B,EPAS1,HRAS,NF1和MAML3.3中的体质突变.

结论:

较高的COPPS分数 (≥3) 与PPGL转移/复发风险增加相关.
COPPS是一种敏感和特定的工具,用于预测PPGL转移/复发.
联合使用COPPS,SDHB和S-100蛋白表达可以增强PPGL的风险分层.

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