VHL and neoplasm: Male mice carrying VHL conditional knockout present abnormalities in testis development, reduced sperm count, impaired spermatogenesis and infertility, besides altered vascularization in multiple organs which is a conventional marker of nonfunctional pVHL [19]; 25%–60% of VHL male patients develop epididymal papillary cystadenomas [20,21], an otherwise rare form of neoplasm affecting male reproductive organs [22].