The von Hippel-Lindau<unk>  suppressor gene generally has a germline mutation. This suppressor gene is also called elongin binding protein and G7 protein. The VHL protein is involved in up-regulation of hypoxic response via the hypoxia inducible factor HIF-1 alpha. Mutations generally prevent the production of any functional VHL protein or result in a change of structure of VHL protein. This genetic disorder shows an autosomal dominant inheritance pattern, with about 20% of patients possessing a new mutation. There are usually several other<unk> s which are part of the syndrome, including<unk> s of the central nervous system, kidneys, pancreas, adrenal glands, epididymis, broad ligament, along with the<unk> <unk> . The vast majority of patients with an<unk>  have von Hippel-Lindau syndrome.
Endolymphatic sac tumor
Nabothian cyst
Gonadoblastoma
Endodermal sinus tumor
Teratoma
Embryoma
Theca lutein cyst
Luteoma