This prevalence is lower than that of VHL-CNS-Hb (1.12 patients per 100,000) but higher than that of VHL-pNET (0.12 patients per 100,000) estimated in the current study, which is generally consistent with the proportion of VHL patients that develops each type of tumor (CNS-Hb: 70-80%, RCC: 25-70%; pNET: 9-17%) [1, 8]. Here, VHL is linked to von Hippel-Lindau disease.