Table 1.

Genetic and clinical characteristics of new genes or new presentations of known genes associated with PGL development.

Gene	Inheritance	Locus	Associated tumors/features
DNMT3A	Autosomal dominant	2p23.3	Gain-of-function mutations: H&N PGL
DLST	Autosomal dominant	14q24.3	PGL (multiple) >> PCC
SUCLG2a	ND	3p14.1	PCC >>>> pPGL
MAML3 fusions	Sporadic	4q31.1	PCC
RET fusions	Sporadic	10q11.21	PCC

^aMore evidence are needed before considering it as a susceptibility gene.