Zhou Y, Zhang W Q, Chen Y H, Cui Y Y, Wang Y, Li T Y, Chang X Y, Tong A L
Department of Endocrinology, Key Laboratory of Endocrinology, National Health Commission of the People's Republic of China, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.
Department of Endocrinology, Key Laboratory of Endocrinology, National Health Commission of the People's Republic of China, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China Graduate School, Hebei North University, Zhangjiakou 075000, China.
Zhonghua Yi Xue Za Zhi. 2024 Jul 23;104(28):2613-2618. doi: 10.3760/cma.j.cn112137-20231218-01404.
To explore the correlation between clinical characteristics and pathological features in patients with pheochromocytoma/paraganglioma (PPGLs). A case series study. A retrospective analysis was conducted on patients with single and primary PPGLs after postoperative pathological diagnosis who were admitted to Peking Union Medical College Hospital between January 2019 and December 2022. The patients were divided into the Ki-67<3% group and the Ki-67≥3% group with Ki-67 proliferation index of 3% as the threshold. The relationship between clinical and pathological characteristics of PPGLs was analyzed. A total of 399 PPGLs patients were included, with 177 males and 222 females, aged [(, )] 45.0(35.5, 53.0) years. Among them, 226 (56.6%) cases originated from the adrenal gland, while 104 cases (26.1%) from the retroperitoneum. 20.9% (27/129) of the patients were found to harbor germline mutations of susceptibility genes, with SDHB mutations being the most common (10.1%, 13/129). The Ki-67 staining was performed on 302 cases, with a Ki-67 proliferation index [(, )] of 2.0% (1.0%, 3.0%). There were 194 cases in Ki-67<3% group and 108 cases in Ki-67≥3% group. Compared with the patients in Ki-67<3% group, the age of onset in Ki-67≥3% group was younger (=0.029). Compared with the patients with paragangliomas without SDHB or Cluster 1A-related gene mutations, positive I-meta-iodobenzylguanidine (I-MIBG) imaging or negative O-6-methylguanine-DNA methyltransferase (MGMT) immunohistochemistry staining, those with SDHB or Cluster 1A-related gene mutations, negative I-MIBG imaging or positive MGMT immunohistochemistry staining had a higher Ki-67 index (all <0.05). Compared with adrenal pheochromocytoma, retroperitoneal paragangliomas had a higher proportion of SDHB mutations and a higher proportion of normetanephrine (NMN) secretory types (all <0.05). Compared with adrenal pheochromocytoma, the maximum diameter of head and neck paraganglioma tumors was smaller [3.0 (1.9, 3.8) cm vs 4.7 (3.4, 6.4) cm, <0.001] and the proportion of Ki-67≥3% was higher (61.3% vs 33.8%, =0.007). PPGLs patients with earlier onset age, SDHB or Cluster 1A-related gene mutations, negative I-MIBG imaging, or positive MGMT immunohistochemistry staining tend to have a higher Ki-67 index. Head and neck tumors, though smaller, exhibit a higher proliferation potential.
探讨嗜铬细胞瘤/副神经节瘤（PPGLs）患者临床特征与病理特征之间的相关性。一项病例系列研究。对2019年1月至2022年12月在北京协和医院住院、术后经病理诊断为单发原发性PPGLs的患者进行回顾性分析。以Ki-67增殖指数3%为界值，将患者分为Ki-67<3%组和Ki-67≥3%组。分析PPGLs临床与病理特征之间的关系。共纳入399例PPGLs患者，其中男性177例，女性222例，年龄为[(, )]45.0（35.5，53.0）岁。其中，226例（56.6%）起源于肾上腺，104例（26.1%）起源于腹膜后。20.9%（27/129）的患者被发现携带易感基因的胚系突变，其中SDHB突变最为常见（10.
