Table 3.

Frequency of lesions and average age range of presentation in VHL patients

Clinical Feature	Average (range) of presentation (years)	Frequency (%)	Reference
CNS hemangioblastoma	30 (9–78)	60–80%	1
Retinal hemangioblastoma	25 (1–67)	49–62%	4, 6
Endolymphatic sac tumors	31 (12–50)	6–15%	7, 15
Renal cell carcinoma or cysts	39 (16–67)	30–70%	6
Pheochromocytoma	30 (5–58)	10–20%	6
Pancreatic neuroendocrine tumors or cysts	36 (1–70)	35–70%	8, 15
Epididymal cystadenomas	Unknown (16–40)	25–60%	6, 15
Broad ligament cystadenomas	Unknown (16–46)	Unknown	8