Our kidneys function in our bodies to remove excess water, salt, and waste products from the blood. There are different areas of the kidneys that play a role in this important job. Our kidneys also help control our blood pressure and make sure we have enough red blood cells in our bodies to stay healthy. Unfortunately, cancer can start in our kidneys, just like any other part of our bodies, and cause problems. And just like many other parts of our bodies that can develop cancer, kidney cancer can be hereditary.
Kidney Cancer Types
There are many types of kidney cancer, but the most common type of kidney cancer is renal cell carcinoma. Approximately 90% of kidney cancers are renal cell carcinomas, and they typically grow as a single tumor within the kidney. Most people diagnosed with kidney cancer are older, typically between the ages of 65 and 74. Kidney cancer is twice as common in men than in women and is more common in African Americans and American Indian / Alaskan Natives.
While all cancers are caused by changes in our DNA inside our cells, some of those changes can be inherited. The great majority of cancers are sporadic, meaning the changes in our DNA were caused by random chance or our environment. However, 5-10% of cancers are inherited, passed down from generation to generation. There are several inherited cancer syndromes that are associated with cancer of the kidney. If you have a family history of kidney cancer, it may be possible that you have an inherited cancer syndrome. There is genetic testing available to determine if the cause of the kidney cancer in your family is an inherited change in your DNA.
Hereditary Leiomyomatosis and Renal Cell Carcinoma is an inherited cancer syndrome that is caused by a change in DNA in a gene called FH. This syndrome can cause firm reddish skin bumps called leiomyomas, which show up around the age of 25. For women, leiomyomas can also appear in the uterus as fibroids, which can cause pain and problems with menstruation. In addition to leiomyomas, there is an increased risk of kidney cancer, specifically renal cell carcinoma. Individuals with this cancer syndrome tend to develop cancer in the kidneys at younger ages, in their 40s, and the cancer is typically more aggressive. Families who are found to have a mutation in the FH gene can inherit the gene in an autosomal dominant pattern, which means each family member has a 50% chance of inheriting the mutated gene.
Birt-Hogg-Dube syndrome is caused by a change in DNA in a gene called FLCN. This syndrome often also involves a wide variety of skin manifestations, such as white bumps on the skin called fibrofolliculomas. Individuals can also have lung cysts, which causes a high risk for spontaneous pneumothorax. There is also an increased risk of kidney cancer, and individuals tend to be diagnosed also at younger ages in their 40s. This syndrome is also inherited in an autosomal dominant pattern.
Kidney Cancer Diagnosis
Von Hippel-Lindau syndrome is caused by a change in DNA in a gene called VHL. This syndrome places an individual at increased risk for a benign, highly vascular tumor called a hemangioblastoma, which can happen in the brain, spinal cord, and retina. There are also benign tumors associated with this syndrome, including neuroendocrine tumors, endolymphatic sac tumors (base of the skull), and pheochromocytomas (adrenal gland). It’s also possible to develop cysts in the kidneys, pancreas, and testicles. Cancer of the kidney also occurs in about 70% of individuals diagnosed with Von Hippel-Lindau syndrome. This syndrome is also inherited in an autosomal dominant pattern.
It is important to remember that even if your family has a mutation in one of these genes, it does not necessarily mean you will develop kidney cancer. There are also screening options to look for cancer at the early stages or before it develops. If you are interested in learning more about your risks of kidney cancer, or are interested in pursuing genetic testing for inherited cancer syndromes, please contact AT-GC to meet with a genetic counselor.