Surgical Approaches for Clear Cell Renal Cell Carcinoma in von Hippel-Lindau

The incidence of renal cell carcinoma attributable to hereditary causes is ~4–6%. Of these, von Hippel-Lindau (VHL) is the most common. As a tumor suppressor gene, VHL plays a crucial role in limiting angiogenesis and by extension neoplasia. In the last 2 decades, over 1500 germline mutations have been identified in the VHL gene located on the short arm of chromosome. Although, VHL patients also experience tumors such as central nervous system and retinal hemangioblastomas, pheochromocytomas, and endolymphatic sac tumors, their disease related mortality used to be due to renal cell carcinoma. Timely surgical intervention using minimal invasive techniques and nephron sparing surgeries for small renal masses have led to improved survival rates among VHL patients.