嗜铬细胞瘤/副神经节瘤免疫微环境

Immune microenvironment of pheochromocytomas and paragangliomas

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。

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.