Von Hippel-Lindau (VHL) disease is the most common type of hereditary renal cell carcinoma in urology. The affected members in VHL disease families are at risk for developing tumors in various organs such as central nervous system, kidney, eye, and ear beginning from various ages, sometimes leading to the difficulties in recognition of this tumor syndrome. Currently, this tumor syndrome is becoming widely known by Chinese urologists. Many cases have been reported in China, but only a few reports have been found in the literature to summarize the clinical and molecular characteristics in Chinese VHL patients. Editorial CommentUrologyVol. 83Issue 3PreviewPatients with Von Hippel-Lindau (VHL) disease are at risk for the development of tumors in a number of locations, including the kidneys (clear cell renal cell carcinoma), adrenals (pheochromocytomas), central nervous system (hemangioblastomas), eyes (retinal angiomas), pancreas (pancreatic neuroendocrine tumors), ears (endolymphatic sac tumors), and epididymis (cystadenomas). Although the gene for this autosomal dominant inherited renal cancer disorder was identified in 1993, we still do not know why patients develop tumors in some organs and not others. Full-Text PDF