Clear cell renal cell carcinoma (ccRCC) is a type of kidney cancer that causes tumors to grow inside your kidneys. ccRCC tumors look clear under a microscope, giving this cancer its name. This type of cancer is the most common type of renal cell carcinoma in adults. According to the National Cancer Institute (NCI), it makes up about 80 percent of all renal cell carcinoma cases in the United States.

Studies are still being done to help experts fully understand the causes of ccRCC. Right now, the exact causes aren’t clear.

However, the NCI says that many cases of ccRCC are found in families with a hereditary genetic condition called Von Hippel-Lindau (VHL) syndrome. This condition causes mutations on the VHL gene.

Mutations to the VHL gene appear to cause ccRCC. But researchers still need to determine how this mutation happens and why it causes ccRCC.

The American Cancer Society (ACS) says that in addition to mutations on the VHL gene, some risk factors increase your chance of ccRCC. Having one or more ccRCC risk factors doesn’t mean you will develop ccRCC. But they do increase your chances, especially if you have a family history.

Risk factors for ccRCC include:

  • Smoking. Smokers are at a higher risk of developing most types of cancer, including ccRCC. If you smoke, quitting can lower your risk.
  • High blood pressure. People with high blood pressure are at increased risk of all types of kidney cancer, including ccRCC.
  • Workplace trichloroethylene exposure. A 2012 review showed that exposure to trichloroethylene and other toxic substances in the workplace can increase the risk of ccRCC.
  • Obesity. Obesity can increase your risk of ccRCC.
  • Advanced kidney disease. Advanced kidney disease and dialysis treatment can increase your risk of ccRCC.
  • Cowden syndrome. People with Cowden syndrome are at higher risk for cancers of the breast, thyroid, and kidney, including ccRCC.
  • Birt-Hogg-Dubé (BHD) syndrome. BHD syndrome leads to an increased risk of kidney tumors, including ccRCC.
  • Tuberous sclerosis. This condition generally causes benign tumors to form but can occasionally cause ccRCC tumors.
  • Gender and race. All types of renal cancer are more common in men and Black Americans. However, this might be linked to a higher likelihood of other risk factors such as high blood pressure, smoking, and workplace trichloroethylene exposure in those groups.

The ACS stages ccRCC using a system called the TNM system, developed by the American Joint Committee on Cancer (AJCC). Staging ranges between 1 and 4 and is based on:

  • Tumor size (T). This measures how far the tumor has grown and spread.
  • Lymph node spread (N). This tells you if the cancer has spread to lymph nodes.
  • Metastasis (M). This tells you if the cancer has spread through the body.

The lower the ccRCC stage, the less the cancer has spread. You can check out the chart below for a breakdown of ccRCC staging.

ccRCC stageWhat it means
stage 1 (T1, N0, M0)Tumor is less than 7 centimeters in size and only in one kidney. No spread to lymph nodes or other organs.
stage 2 (T2, N0, M0)Tumor is larger than 7 centimeters in size but still only in one kidney. No spread to lymph nodes or other organs.
stage 2 (T3, N0, M0)Tumor has spread to a major vein or the tissue around the kidney. No spread to lymph nodes or other organs.
stage 3
(T1 through T3, N1, M0)		Tumor can be any size as long as it has not spread beyond the renal fascia. Cancer has spread to nearby lymph nodes. No spread to distant lymph nodes or to other organs.
stage 4 (T4, any N, M0)Tumor is growing outside of kidneys and might be growing into adrenal glands. Cancer might have spread to lymph nodes. No spread to other organs.
stage 4 (any T, any N, M1)Tumor can be of any size, cancer might have spread to lymph nodes. Cancer has spread to other organs.

Treatment for ccRCC will depend on the stage of cancer at diagnosis. The NCI says that treatment options generally include surgery, ablation, immunotherapy, radiation, and targeted therapy. Your doctor will discuss the best path for you once your ccRCC has been confirmed and staged.

  • Surgery. Surgery to remove the tumor is often the first treatment for ccRCC. You might have the part of your kidney around the tumor or even your entire kidney removed, depending on the size of the tumor.
  • Ablation. Ablation procedures can destroy some tumors without surgery and are a good option for some people.
  • Radiation therapy. Radiation therapy can kill tumor cells with radiation.
  • Immunotherapy. Immunotherapy is a type of biological therapy that strengthens your immune system and helps it destroy cancer cells.
  • Targeted therapy. A class of medications called tyrosine kinase inhibitors is used to help block signaling pathways in the cancerous cells, slowing their growth.

Your medical team will develop a treatment plan to help you fight ccRCC, but there are some very important parts of your care that can’t happen in an operating room.

Managing your everyday life with ccRCC can feel like a huge challenge. But there are some simple steps you can take at home, with friends, and with other healthcare professionals. Great ways to take care of yourself during treatment include:

  • Eating healthier. There isn’t a set diet plan for ccRCC, but eating healthy can help you feel better during treatments and recovery. Talk with your doctor before making any major changes to your diet.
  • Taking time for mental health. It can be hard to talk about a cancer diagnosis, even with family and friends. Mental health professionals such as counselors and psychologists can help you sort through the stress and emotions of managing ccRCC.
  • Asking for help. It’s a good idea to turn to friends, family, loved ones, or other supportive people when you’re managing ccRCC. Local charities and cancer support groups can help you find support if you’re in need.

Getting help

When you need support during your cancer treatment, there are places you can turn to. Beyond friends and family, you can reach out to:

  • The Kidney Cancer Association. You’ll find resource videos, peer support groups, and a helpful patient liaison program on the Kidney Cancer Association website.
  • The Cancer Support Helpline. If you’re looking for live support by phone or live chat, check out the Cancer Support Helpline. Support is available in over 200 languages to connect you with local resources, support groups, and more.
  • Smart Patients Kidney Cancer Forum. You and your family can share your thoughts, frustrations, questions, successes, and more on the Smart Patients message boards.

What’s the recurrence rate of clear cell renal cell carcinoma?

The likelihood of recurrence, or the cancer coming back, depends on the stage at diagnosis. Studies have found that people with ccRCC have an average 30 percent recurrence rate following surgery.

Is clear cell renal cell carcinoma malignant?

Yes, ccRCC is a malignant tumor. ccRCC that is not treated will spread to other parts of the body and can be fatal.

Is clear cell renal cell carcinoma aggressive?

The growth of cancer cells in ccRCC can be either slow or fast depending on the individual. However, ccRCC often responds very well if it is diagnosed and treated early.

What are the symptoms of clear cell renal cell carcinoma?

Some people with ccRCC don’t have any noticeable symptoms at all. When symptoms do occur, they include:

  • fever
  • unintentional weight loss
  • pain
  • fatigue
  • a lump on your side

The outlook following any diagnosis of ccRCC depends on the stage at diagnosis, your overall health, and how well you respond to treatment. Currently, the NCI reports that the 5-year survival rate for ccRCC is 50 to 69 percent. However, cancer treatments continue to improve, and survival rates are likely to increase in the years to come. Additionally, the outlook is much better for people who are diagnosed at early stages when ccRCC can often be treated and cured.

Clear cell renal cell carcinoma is the most common type of renal cell carcinoma. It causes tumors made up of clear cells to grow inside your kidneys. Often, the first treatment for ccRCC is to remove the tumor with surgery or ablation. Treatments such as immunotherapy and targeted therapy can be used along with surgery. The outlook for people with ccRCC is good with prompt diagnosis and treatment.