Table 2.

Clinical Characteristics of Patients with PPGL According to the Molecular Clusters

Characteristic	Molecular clusters (total n=28)			P value
	Pseudohypoxic TCA cycle-related (n=11)	Pseudohypoxic VHL/EPAS1-related (n=6)	Kinase-signaling (n=11)
Age, yra	47.6±16.7	45.7±11.9	45.0±15.9	0.920
Age of onset, yra	43.2±18.7	35.8±15.3	37.8±13.8	0.616
Female sex	5 (45.5)	4 (66.7)	7 (63.6)	0.599
Type of PPGL
PCC	4 (36.4)	5 (83.3)	11 (100.0)	0.003
PGL	7 (63.6)	1 (16.7)	0
Family history	0	2 (33.3)	7 (63.6)	0.006
Location
PCC (n=20)
Unilateral adrenal gland	4 (100.0)	3 (60.0)	7 (63.6)	0.339
Right adrenal gland	2 (50.0)	1 (20.0)	5 (45.5)
Left adrenal gland	2 (50.0)	2 (40.0)	2 (18.2)
Bilateral adrenal glands	0	2 (40.0)	4 (36.4)
PGL (n=8)
Head and neck	1 (14.3)	0	0	0.686
Othersb	6 (85.7)	2 (100.0)	0
Tumor size, cm (n=23)c	4.1 (3.1–4.7)	3.5 (2.0–4.2)	3.4 (2.0–4.7)	0.688
Metastasis	7 (63.6)	3 (50.0)	0	0.006
Synchronous	2 (18.2)	2 (66.7)	0	0.260
Metachronous	5 (45.5)	1 (33.3)	0
Recurrence	4 (36.4)	2 (33.3)	1 (9.1)	0.292
Presence of other tumors	1 (9.1)	3 (50.0)	8 (72.7)	0.010
Discovery route (n=24)
Incidental	2 (25.0)	5 (83.3)	4 (40.0)	0.086
Symptomatic	6 (75.0)	1 (16.7)	4 (40.0)
Gene mutation	0	0	2 (20.0)
Biochemical profile
Adrenergic/Noradrenergic	0	0	4 (36.4)	0.036
Adrenergic	0	0	1 (9.1)
Noradrenergic	9 (81.8)	3 (50.0)	2 (18.2)
Silent	2 (18.2)	3 (50.0)	4 (36.4)

Values are expressed as mean±standard deviation, number (%), or median (interquartile range).

PPGL, pheochromocytoma and paraganglioma; TCA, tricarboxylic acid; VHL, von Hippel-Lindau; EPAS1, endothelial PAS domain-containing protein 1; PCC, pheochromocytoma; PGL, paraganglioma.

^aP valuewas calculated by one-way analysis of variance, as they were parametric variables (Shapiro-Wilk test P=0.699 for age; P=0.461 for age of onset);

^bOthers include retroperitoneum, pancreas, para-aortic space, and bladder;

^cP value was calculated by the Kruskal-Wallis test, as it was a nonparametric variable (Shapiro-Wilk test P<0.001).