Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors originating from chromaffin cells in the adrenal medulla (PCCs) or extra-adrenal sympathetic or parasympathetic paraganglia (PGLs). About 40% of PPGLs result from germline mutations and therefore they are highly inheritable. Although dysfunction of any one of a panel of more than 20 genes can lead to PPGLs, mutations in genes involved in the VHL/HIF axis including
Frontiers in Endocrinology
Peng, Song; Zhang, Jun; Tan, Xintao; Huang, Yiqiang; Xu, Jing; Silk, Natalie; Zhang, Dianzheng; Liu, Qiuli; and Jiang, Jun, "The VHL/HIF Axis in the Development and Treatment of Pheochromocytoma/Paraganglioma" (2020). PCOM Scholarly Papers. 2098.