Table 1.

Newly discovered in the pathogenesis of PPGLs.

Gene	Year of Discovery	Pathophysiology	Gene Type	Metabolomics	References
CSDE1	2016	Tumor suppressor gene involved in mRNA stability and cellular apoptosis	Somatic	Adrenergic	[6,7]
H3F3A	2016	Encodes histone H3.3 protein that regulates chromatin formation	Somatic	NA	[35,36]
MET	2016	MAPK signaling pathway	Germline, somatic	NA	[23]
MERTK	2016	Tyrosine kinase receptor	Germline	NA	[11,37,38]
UBTF-MAML3	2017	Unique methylation profile mRNA overexpression involved in Wnt receptor and hedgehog signaling pathways	Fusion	Adrenergic	[6,39]
SLC25A11	2018	Encodes malate-oxalate carrier protein of malate-aspartate shuttle	Germline	Noradrenergic	[40,41]
IRP1	2018	Cellular iron metabolism regulation	Somatic	noradrenergic	[42]
DLST	2019	Encodes E2 subunit of mitochondrial α -KG complex which converts α-KG to succinyl-CoA	Germline	Noradrenergic	[23,43]
SUCLG2	2021	Catalyzes conversion of succinyl-coA and ADP/GTP to succinate and ATP/GTP	Germline	Noradrenergic	[44]