Basic & Clinical Medicine ›› 2024, Vol. 44 ›› Issue (6): 742-747.doi: 10.16352/j.issn.1001-6325.2024.06.0742

• Special Issues:Pathogenesis of Adrenal Tumors • Previous Articles     Next Articles

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

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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