基础医学与临床 ›› 2024, Vol. 44 ›› Issue (6): 742-747.doi: 10.16352/j.issn.1001-6325.2024.06.0742

• 特邀专题:肾上腺肿瘤的发病机制 • 上一篇    下一篇

嗜铬细胞瘤/副神经节瘤免疫微环境

周玥, 童安莉*   

  1. 中国医学科学院 北京协和医学院 北京协和医院 内分泌科 国家卫生健康委员会内分泌重点实验室,北京 100730
  • 收稿日期:2024-03-31 修回日期:2024-04-23 出版日期:2024-06-05 发布日期:2024-05-24
  • 通讯作者: *tonganli@hotmail.com
  • 基金资助:
    中央高水平医院临床研究专项(2022-PUMCH-C-028);中国医学科学院医学与健康科技创新工程(2021-I2M-C&T-B-002)

Immune microenvironment of pheochromocytomas and paragangliomas

ZHOU Yue, TONG Anli*   

  1. Department of Endocrinology,Key Laboratory of Endocrinology of National Health Commission,Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100730,China
  • Received:2024-03-31 Revised:2024-04-23 Online:2024-06-05 Published:2024-05-24
  • Contact: *tonganli@hotmail.com

摘要: 嗜铬细胞瘤/副神经节瘤(PPGLs)是罕见的神经内分泌肿瘤,遗传易感性高,其中SDHx突变为主的假性缺氧型肿瘤恶性潜能大。约10%~17%的PPGLs肿瘤会发生转移,转移性PPGLs缺乏有效的治疗,预后差。免疫微环境与肿瘤的发生发展密切相关,能够预测患者预后及其对免疫检查点抑制剂治疗的反应。目前已有部分研究初步描绘了PPGLs免疫图谱。本文主要就PPGLs免疫微环境中各类免疫细胞浸润情况、免疫检查点分子的表达及其与患者基因背景及肿瘤转移的关系进行综述,以更好地理解肿瘤免疫逃逸机制,并为转移性PPGLs患者寻找新的治疗方案提供思路。

关键词: 嗜铬细胞瘤/副神经节瘤, 转移性嗜铬细胞瘤/副神经节瘤, 肿瘤免疫微环境, 免疫细胞, 免疫检查点

Abstract: Pheochromocytomas/Paragangliomas(PPGLs) are rare neuroendocrine tumors with a high hereditary predisposition. The pseudo-hypoxic PPGLs dominated by SDHx mutations show a higher potential of metastasis. Approximately 10%-17% of PPGLs develop metastasis and patients with metastatic diseases have restricted treatment options and a poor prognosis. Tumor immune microenvironment (TIME) plays a crucial role in the development and progression of tumors as well as predicting patients’ prognosis and their response to immune checkpoint inhibitors. Several studies have initially characterized the immune landscape of PPGLs. This review focuses on the infiltration of immune cells,the expression of immune checkpoints in the TIME of PPGLs and their relationships with genetics and metastasis of tumors in order to better understand the mechanisms of tumor immune evasion in PPGLs and provide insights to support novel treatment strategy for metastatic PPGLs.

Key words: pheochromocytomas/paragangliomas, metastatic pheochromocytomas/paragangliomas, tumor immune microenvironment, immune cell, immune checkpoint

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