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MyPART - My Pediatric and Adult Rare Tumor Network
 

Clear Cell Renal Cell Carcinoma

What is clear cell renal cell carcinoma? 

Clear cell renal cell carcinoma, or ccRCC, is a type of kidney cancer. The kidneys are located on either side of the spine towards the lower back. The kidneys work by cleaning out waste products in the blood. Clear cell renal cell carcinoma is also called conventional renal cell carcinoma. 

Clear cell renal cell carcinoma is named after how the tumor looks under the microscope. The cells in the tumor look clear, like bubbles.

How common is ccRCC?

In adults, ccRCC is the most common type of kidney cancer, and makes up about 80% of all renal cell carcinoma cases. ccRCC is more common in adults than children. Renal cell carcinoma makes up 2-6% of childhood and young adult kidney cancer cases. 

How is ccRCC diagnosed? 

Patients with ccRCC may have pain or feel tired. Sometimes, patients do not have any noticeable symptoms. Symptoms can include:

  • Blood in the urine
  • Pain
  • Weight loss
  • Feeling tired
  • Fever
  • A lump in the side

For people without symptoms, these tumors can be discovered if the person has an imaging test for another reason.

Imaging: If are suspected to have clear cell renal cell carcinoma, your doctor will use imaging scans such as X-rays, CT or MRI to look at the size of the tumor. They will also check for signs that the tumor has spread to other parts of the body. 

Biopsy: To check if the tumor is ccRCC your doctor will perform a biopsy, taking a small sample from the tumor with a needle. An expert, called a pathologist, will study cells from the sample under the microscope to see what kind of tumor it is.

How is ccRCC treated?

Treatments for people with ccRCC include surgery and immunotherapy. Treatment will depend on how much the cancer has grown.

Surgery: Once ccRCC is diagnosed, you may have surgery to remove the cancer and part of the kidney surrounding it. In early stage ccRCC, part of the kidney with the cancer is taken out. If ccRCC is in the middle of the kidney, or if the tumor is large, sometimes the entire kidney must be removed. In later stage ccRCC, removal of the kidney is controversial but may be appropriate in some patients. 

Immunotherapy: Immunotherapy helps the body’s immune system fight the cancer cells. 

Targeted therapy: Targeted therapy targets the changes in cancer cells that help them grow, divide, and spread. Some targeted therapies that are used to treat clear cell renal carcinoma include cabozantinib, axitinib, sunitinib, sorafenib, and pazopanib. 

Other treatments can be used that do not involve removing the kidney, such as:

  • Radiation therapy, which uses radiation to kill the tumor cells
  • Thermal ablation, which uses heat to kill the tumor cells
  • Crysosurgery, which uses liquid nitrogen to freeze and kill the tumor cells

Does ccRCC run in families? 

ccRCC can run in families.  Almost all cases of ccRCC that run in families are found in people with a genetic condition called Von Hippel-Lindau syndrome, but other hereditary conditions may also be associated with ccRCC. People with Von Hippel-Lindau syndrome have mutations in the VHL gene.

How does ccRCC form?

Scientists are always working to understand how cancer forms, but it can be hard to prove. Because ccRCC can run in families, we know that changes in the VHL gene are important in causing ccRCC. The VHL gene is also changed in ccRCC from people without a family history of Von Hippel-Lindau syndrome. Scientists have learned a lot about what the VHL gene does in the body. This has given scientists clues about treatments to try for ccRCC.

What is the prognosis for people with ccRCC? 

The estimate of how a disease will affect you long-term is called prognosis. Every person is different and prognosis will depend on many factors, such as

  • Where the tumor is in your body
  • If the cancer has spread to other parts of your body
  • How much of the tumor was taken out during surgery

If you want information on your prognosis, it is important to talk to your doctor. NCI also has resources to help you understand cancer prognosis.

Doctors estimate ccRCC survival rates by how groups of people with ccRCC have done in the past. Because there are so few pediatric ccRCC patients, these rates may not be very accurate. They also don’t take into account newer treatments being developed. 

With this in mind, ccRCC patients with smaller tumors have a better chance of survival than patients with larger tumors. The 5-year survival rate for patients with ccRCC is 50-69%. When ccRCC is already large or has spread to other parts of the body, treatment is more difficult and the 5-year survival rate is about 10%.