Kidney Cancer is not just one disease. There are a number of different cell types, each of which responds somewhat differently to treatment. Dr. W. Marston Linehan and his team at the U.S. National Cancer Institute has done extensive work in understanding the different kinds of kidney cancer.
- Clear cell = 75%
- Papillary type I = 5%
- Papillary type II = 10%
- Chromophobe = 5%
- Oncocytoma = 5%
In each case, a different genetic alteration is present, which changes the chemical processing in the cell and makes it more or less responsive to the action of each of the drugs on the market. Thus the genetic analysis of the tumor tissue may be important in choosing the right drug.
In addition, if a “germline” mutation is present in every cell of this patient, there may be more than just kidney cancer to be concerned about. Approximately 5-10% of people with kidney cancer may have a hereditary condition, and the percentages are higher if the patient is younger than age 50. The tumor tissue type may be helpful in determining what genes should be screened for in genetic testing, and what other organ systems may be at risk for medical issues. If one of these syndromes is present, it will influence the optimal choice of treatment for this patient, and influence a plan of surveillance to watch for other issues.
- Clear cell — screen for changes in the VHL gene, implicated in von Hippel-Lindau (VHL)
- Papillary type 1 — screen for changes in the MET proto-oncogene, implicated in Hepatocellular Carcinoma (HCC) and Hereditary Papillary Renal cell carcinoma (HPRCC).
- Papillary type 2 — screen for changes in the FH gene, implicated in Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC)
- Chromophone — screen for changes in the FLCN gene, implicated in Birt-Hogg-Dubé syndrome (BHD).
- Oncocytoma — screen for changes in the FLCN gene, implicated in Birt-Hogg-Dubé syndrome (BHD).
The new 2016 WHO Classification of Tumors of the Urinary System and male genital organs is now available. It contains the new kidney tumors that have recently been described at conferences, and will help to standardize terminology in the design of studies monitoring response to therapy and clinical outcome.
Linehan WM et al, “Genetic Basis of Cancer of the Kidney: Disease-Specific Approaches to Therapy,” Clin Cancer Res September 15, 2004 10; 6282S – http://clincancerres.aacrjournals.org/content/10/18/6282S.abstract
Linehan WM et al, “Genetic basis of kidney cancer: role of genomics for the development of disease-based therapeutics.” Genome Res 2012, 22:2089-100 – http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3483538/