Of all renal cancer in the general population, 75% is clear cell, 15% is papillary, 5% is chromophobe, 5% is unclassified, and 1% occurs in the collecting duct. Only about 5-10% of all kidney cancer is due to a germline (heritable) genetic change.

VHL is the leading heritable cause of clear cell renal cancer. While heritable VHL accounts for only 5% of all clear cell RCC, changes in the VHL gene that happen during one's lifetime contribute to the growth of more than 60% of all clear cell RCC.

People with HLRCC have mostly papillary kidney cancer, sometimes in the collecting duct. These tumors are caused by a change in the Fumarate Hydratase (FH) gene, which also affects HIF production.

People with Birt-Hogg-Dubé syndrome, another heritable renal cancer, can have multiple types of renal cancer, even in the same kidney. Mutations in the Folliculin (FLCN) gene disrupt another tumor-suppressor function.

Through study of these and other familial forms of renal cancer we are learning more about the pathway to kidney cancer in the cell.

As printed in the VHL Family Forum 14:1, April/May 2006. For permission to reprint, please contact VHL Family Alliance, editor@vhl.org. Further information is available from the VHL Family Alliance, info@vhl.org.