Table 1.

Meta-analytical results on the frequency of pheochromocytoma and/or paraganglioma (PPGL) among patients with von Hippel–Lindau disease (vHL).

	N studies	N vHL	Frequency (%, 95% CI)	Q Cochran P value	I²
Participants with vHL (all vHL types)
Frequency of participants with PPGL	45	4263	19.4 (15.9–23.6)	<0.001	86.1%
Participants with bilateral pheochromocytoma	14		59.5 (47.0–70.9)	0.611	0%
Males with PPGL	15		58.3 (47.6–68.4)	0.837	0%
Participants with vHL type 2		840
Frequency of participants with PPGL	52		60.0 (53.4–66.3)	<0.001	54.6%
Participants with PPGL in vHL type 2A	28	411	58.2 (49.7–66.3)	0.010	36.2%
Participants with PPGL in vHL type 2B	24	256	49.8 (39.9–59.7)	0.013	42.7%
Participants with PPGL in vHL type 2C	11	52	84.1 (75.1–93.1)	0.932	0%