Clear Cell Renal Cell Carcinoma

Overview

What is clear cell renal cell carcinoma?

Clear cell renal cell carcinoma (ccRCC) is a subtype of renal cell carcinoma, a kidney cancer. Its name comes from the cancer cells, which look like clear soap bubbles under a microscope.

ccRCC cancer cells rapidly multiply, forming one or more tumors. This cancer typically only affects one kidney but can affect both.

What is renal cell carcinoma (RCC)?

Renal cell carcinoma, or RCC, is the most common kidney cancer. RCC accounts for approximately 9 out of 10 kidney cancer diagnoses. Renal is the Latin word for kidney. Carcinoma is the medical term for cancer that begins in the cells that cover or line an organ.

RCC develops in the cells lining the small tubes (tubules) that help your kidneys filter waste from the blood. The urinary system removes this waste from the body. You may also hear the terms renal cell cancer or renal cell adenocarcinoma.

How common is clear cell renal cell carcinoma (ccRCC)?

ccRCC makes up about 8 in 10 renal cell carcinomas. This makes ccRCC the most common type of renal cell carcinoma and kidney cancer. About 76,000 people in the U.S. get a kidney cancer diagnosis each year.

Who is at risk for clear cell renal cell carcinoma?

Clear cell renal cell carcinoma affects all ages, races and genders. However, it affects twice as many men as women and most commonly appears in people between 50 and 70 years old. Fewer than 6% of cases occur in children or young adults.

Symptoms and Causes

What causes clear cell renal cell carcinoma?

Most kidney cancers like clear cell renal cell carcinoma develop for no known reason. But certain factors may increase your risk, such as:

Is clear cell renal cell carcinoma inherited?

Having a sibling with renal cell carcinoma increases your risk of kidney cancer. Experts aren’t sure why.

Some families have genetic conditions that predispose them to clear cell renal cell carcinoma. These conditions include Von Hippel-Landau (VHL) disease and tuberous sclerosis complex. With these conditions, an inherited gene change or mutation causes cysts and tumors to form. These cysts and tumors are usually not cancerous (benign). But ccRCC can occur in these families, often at younger ages.

What are clear cell renal cell carcinoma symptoms?

Clear cell renal cell carcinoma usually won’t cause noticeable symptoms in its early stage. As the disease progresses, you may experience:

Diagnosis and Tests

How is clear cell renal cell carcinoma diagnosed?

Kidney cancer rarely causes symptoms. That’s why healthcare providers discover more than half of kidney tumors while looking for a cause of other conditions. They may see a tumor on imaging tests like MRIs or CT scans to check for problems like kidney stones.

While imaging tests can detect a tumor, they can’t show whether the mass is benign or malignant. For an accurate diagnosis, you’ll undergo a biopsy or surgery to remove the tumor.

After surgery, healthcare providers take a biopsy of the tumor tissue. The results can confirm a cancer diagnosis and determine whether it’s the clear cell subtype. As many as 4 in 10 small kidney tumors (<4 centimeters) turn out to be noncancerous tumors like renal oncocytomas.

Management and Treatment

How is clear cell renal cell carcinoma treated?

Treatments for ccRCC include:

  • Surgery to remove the cancerous part of your kidney (partial nephrectomy) or your entire kidney (radical nephrectomy).
  • Immunotherapy to engage your body’s immune system to destroy cancer cells. Medications include aldesleukin (Proleukin®).
  • Targeted therapy to stop cancer cells from growing and multiplying. Drugs include sorafenib (Nexavar®) and temsirolimus (Torisel®).
  • Radiation therapy to kill cancer cells with radiation. Similar treatments include cryosurgery (which uses cold temperatures) or thermal ablation (using hot temperatures) to destroy cancer cells.

What are the complications of clear cell renal cell carcinoma?

Clear cell renal cell carcinoma can cause blood clots to form in the renal vein of your affected kidney. The renal vein is the largest blood vessel that carries blood from your kidney to your heart.

Is clear cell renal cell carcinoma aggressive?

Like all carcinomas, ccRCC can spread, or metastasize, to other parts of your body. Metastatic cancer is more difficult to treat. Compared to other kidney cancers, the clear cell type of renal cell carcinoma tends to be more aggressive and spread faster.

Renal cell carcinoma most commonly spreads to the:

Outlook / Prognosis

What is the survival rate for clear cell renal cell carcinoma?

As many as 7 in 10 people with small ccRCC tumors are alive five years after the initial diagnosis. Treatment is less effective on large tumors or metastatic cancer. In those cases, five-year survival rates may drop to about 1 in 10. A cancer prognosis depends on many factors, including:

  • Cancer spread.
  • Effectiveness of treatments.
  • Location, size and number of tumors (cancer stage).

Living With

When should I call the doctor?

Call your healthcare provider if you experience:

  • Blood in your urine.
  • Lump or pain in your side that doesn’t go away.
  • Unexplained fever or weight loss.

What should I ask my healthcare provider?

You may want to ask your healthcare provider:

  • What type of kidney cancer do I have?
  • What is the best treatment for me?
  • What are the treatment side effects?
  • Am I at risk for metastatic cancer? How can I lower this risk?
  • Are other family members at risk for kidney cancer?
  • Should I look out for signs of complications?

Frequently Asked Questions

What is the difference between clear cell renal cell carcinoma (ccRCC) and papillary renal cell carcinoma?

Clear cell and papillary renal cell carcinomas are types of kidney cancer that cause similar symptoms. Treatments are also similar. The main difference is how your kidney cancer cells look under the microscope. Papillary cancer cells resemble long, thin, finger-like growths. Healthcare providers call these growths papillae. About 1 in 10 people with renal cell carcinoma have the papillary type.

A note from Cleveland Clinic

Clear cell renal cell carcinoma (ccRCC) is the most common kidney cancer diagnosis. It can be aggressive and grow faster than other kidney cancers. Surgery, called a nephrectomy, is usually the first step to confirm a diagnosis and treat the cancer. Surgery can remove just the cancerous tumor or your entire diseased kidney. You may also benefit from follow-up therapies like immunotherapy or radiation therapy.

Last reviewed by a Cleveland Clinic medical professional on 01/01/2022.

References

  • American Cancer Society. What Is Kidney Cancer? (https://www.cancer.org/cancer/kidney-cancer/about/what-is-kidney-cancer.html) Accessed 1/1/2022.
  • Genetic and Rare Diseases Information Center. Clear Cell Renal Cell Carcinoma. (https://rarediseases.info.nih.gov/diseases/9574/clear-cell-renal-cell-carcinoma) Accessed 1/1/2022.
  • Merck Manual (Professional Version). Renal Cell Carcinoma. (https://www.merckmanuals.com/professional/genitourinary-disorders/genitourinary-cancer/renal-cell-carcinoma) Accessed 1/1/2022.
  • National Cancer Institute. Clear Cell Renal Cell Carcinoma. (https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-kidney-tumors/clear-cell-renal-cell-carcinoma) Accessed 1/1/2022.
  • Urology Care Foundation. What Is Kidney Cancer? (https://www.urologyhealth.org/urology-a-z/k/kidney-cancer) Accessed 1/1/2022.

Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy