Mutations in vhl can be deletions or missense in the short arm of chromosome 3, resulting in an increased propensity to develop a variety of benign and malignant tumors, such as hemangioblastomas in the central nervous system (CNS), renal cysts and renal cell carcinomas (RCC), pheochromocytomas, pancreatic islet cell tumors, endolymphatic sac tumors, and both broad ligament and epididymal cystadenomas in women and men, respectively (1, 2). Here, VHL is linked to renal cell carcinoma.