Kidney cancer is a disease in which malignant (cancer) cells form in tubules of the kidney.

Kidney cancer (also called renal cell cancer) is cancer that starts in the lining of very small tubes in the kidney called renal tubules. There are two kidneys, one on each side of the backbone, above the waist. Tubules in the kidneys filter and clean the blood. They take out waste products and make urine. The urine passes from each kidney through a long tube called a ureter into the bladder. The bladder holds the urine until it passes through the urethra and leaves the body.

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Cancer that starts in the ureters or the renal pelvis (the part of the kidney that collects urine and drains it to the ureters) is called urothelial cancer. This kind of cancer is different from renal cell cancer and is not associated with the hereditary cancer syndromes described in this summary. Urothelial cancer of the renal pelvis may be associated with another hereditary condition called Lynch syndrome. To learn more, see the health professional summary on Genetics of Colorectal Cancer.

Having certain hereditary syndromes can increase the risk of kidney cancer.

Most of the time, kidney cancer risk is not passed down from parent to child. Kidney cancer that affects multiple generations of a family is called hereditary kidney cancer. Hereditary kidney cancer accounts for only 5%‒8% of all kidney cancers. It is usually linked to a hereditary cancer syndrome. A hereditary cancer syndrome is a disorder in which family members have a higher-than-average risk of developing a certain type or types of cancer. Hereditary cancer syndromes are caused by inherited, harmful genetic changes (also called pathogenic variants or mutations) in certain genes. Hereditary cancer syndromes are sometimes called inherited cancer syndromes or family cancer syndromes. People with the hereditary cancer syndromes described in this summary have an increased risk of kidney cancer.

The hereditary cancer syndromes described in this summary are:

Every person inherits two copies of each gene, one from each parent. These syndromes occur when a person inherits a mutation in one copy of the gene associated with the syndrome. This form of inheritance is called autosomal dominant inheritance.

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Hereditary and non-inherited kidney cancers are different in several ways.

This page provides information about hereditary syndromes associated with kidney cancer. It does not cover information about sporadic kidney cancer or somatic mutations found during tumor sequencing.

Genetic counseling is a communication process between a specially trained health professional and a person concerned about the genetic risk of disease.

It is not always easy to determine whether a condition in a family is inherited. Genetic counselors and other specially trained health professionals can help patients understand their personal and family medical history, their options for genetic testing, and the risks and benefits of learning genetic information. If a patient chooses to get genetic testing, it may be done using a sample of blood, saliva, or skin. Genetic test results can reveal information about other family members and can create tension in the family. Genetic counselors can help people cope with their genetic testing results, including how to discuss the results with family members.

Whether a person should get tested for hereditary kidney cancer depends on certain factors.

Certain clues in the medical history or family history may lead health professionals to think that a person may have a hereditary syndrome. With kidney cancer, people who have one or more of the following criteria may be referred for genetic counseling and testing:

Four hereditary kidney cancer syndromes and the genes that cause them have been identified.

Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC)

HLRCC was considered rare, but it may be one of the most common hereditary cancer syndromes. Some people who have HLRCC may not have symptoms. HLRCC is caused by harmful changes (also called mutations or pathogenic variants) in the FH gene. People with HLRCC could have an increased risk of kidney cancer. The risk may be higher in males with FH mutations. Black people diagnosed with kidney cancer have more FH mutations than people from other racial and ethnic groups. HLRCC is associated with a unique type of kidney cancer that can be fast growing. People with HLRCC may also develop leiomyomas (benign smooth-muscle tumors) in the skin and uterus (fibroids) or paragangliomas (benign tumors near adrenal glands, blood vessels, or nerves).

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The FH gene makes a protein called fumarase. Fumarase helps cells use oxygen and produce energy. When the FH gene is mutated, cells are not able to use oxygen, which may lead to cancer. HLRCC is inherited in an autosomal dominant manner. This means that if one parent has HLRCC, there is a 50% (1 in 2) chance their child will inherit the mutation in the FH gene.

Learn more about HLRCC from the NCATS Genetic and Rare Diseases Information Center.

Von Hippel-Lindau Disease (VHL)

VHL is a rare hereditary syndrome that is caused by harmful changes (also called mutations or pathogenic variants) in the VHL gene. People with VHL have an increased risk of kidney cancer and renal cysts. VHL is associated with a clear-cell type kidney cancer, which is typically slow growing. People with VHL may also develop malignant (cancer) and benign (noncancer) tumors in many parts of the body, including the central nervous system, retina, pancreas, adrenal glands, endolymphatic sac, epididymis (in males), and broad ligament (in females).

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The VHL gene is a type of gene called tumor suppressor gene. Normally, the VHL gene prevents cells from growing and dividing too quickly. When the VHL gene is mutated in certain ways and loses its protective function, uncontrolled cell growth may lead to cancer. VHL is inherited in an autosomal dominant manner. If one parent has VHL, there is a 50% (1 in 2) chance their child will inherit the harmful genetic change in the VHL gene.

Learn more about VHL from the NCATS Genetic and Rare Diseases Information Center.

Birt-Hogg-Dubé Syndrome (BHD)

BHD is a rare hereditary syndrome caused by harmful changes (also called mutations or pathogenic variants) in the FLCN gene. People with BHD have an increased risk of multiple types of kidney cancer that are typically slow growing. People with BHD may also develop skin tumors called fibrofolliculomas, lung cysts, and spontaneous pneumothorax (collapsed lung).

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The FLCN gene is a tumor suppressor gene. Normally, the FLCN gene prevents cells from growing and dividing too quickly. When the FLCN gene is mutated, uncontrolled cell growth may lead to cancer. BHD is inherited in an autosomal dominant manner. This means that if one parent has BHD, there is a 50% (1 in 2) chance their child will inherit the mutation in the FLCN gene.

Learn more about BHD from the NCATS Genetic and Rare Diseases Information Center.

Hereditary Papillary Renal Cancer (HPRC)

HPRC is a rare hereditary syndrome caused by harmful changes (also called mutations or pathogenic variants) in the MET gene. People with HPRC have an increased risk of a type of kidney cancer called papillary kidney cancer, which is typically slow growing. Papillary kidney cancer forms in the cells lining the very small tubes in the kidney called renal tubules.

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The MET gene makes a protein called MET that is involved in cell signaling and growth. When the MET gene is mutated, cells may not respond to signals that normally prevent them from growing, causing cancer to develop. HPRC is inherited in an autosomal dominant manner. This means that if one parent has HPRC, there is a 50% (1 in 2) chance their child will inherit the mutation in the MET gene.

Learn more about papillary kidney cancer from the NCATS Genetic and Rare Diseases Information Center.

About PDQ

Physician Data Query (PDQ) is the National Cancer Institute’s (NCI’s) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.

PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

Purpose of This Summary

This PDQ cancer information summary has current information about the genetics of kidney cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

Reviewers and Updates

Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary (“Updated”) is the date of the most recent change.

The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Cancer Genetics Editorial Board.

Clinical Trial Information

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become “standard.” Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Clinical trials can be found online at NCI’s website. For more information, call the Cancer Information Service (CIS), NCI’s contact center, at 1-800-4-CANCER (1-800-422-6237).

Permission to Use This Summary

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

PDQ® Cancer Genetics Editorial Board. PDQ Hereditary Kidney Cancer Syndromes. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/kidney/patient/kidney-genetics-pdq. Accessed <MM/DD/YYYY>.

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.

Natural History of Renal Cell Carcinoma

The natural history of each RCC syndrome is distinct and influenced by several factors, including histological features and underlying genetic alterations. Although it is useful to follow the predominant reported natural history of each syndrome, each affected individual must be evaluated and monitored for occasional individual variations. The individual prognosis depends on the characteristics of the renal tumor at the time of detection and intervention, which differs for each syndrome (VHL, HLRCC, HPRC, and BHD). Prognostic determinants at diagnosis include the stage of the RCC, whether the tumor is confined to the kidney, primary tumor size, Fuhrman nuclear grade, and multifocality.[14–16]

Family History as a Risk Factor for Renal Cell Carcinoma

Kidney cancer and renal pelvis cancer account for about 3.97% of all adult malignancies in the United States.[1] Epidemiological studies of RCC suggest that a family history of RCC is a risk factor for the disease.[4,17,18] An analysis of individuals with RCCs diagnosed before the year 2000 in the Sweden Family-Cancer Database included all Swedes born since 1931 and their biological parents. The study observed that the risk of RCC was particularly high in the siblings of those with RCC. Siblings of individuals with RCC had a higher relative risk (RR) than parent-child pairs.[17] Further work from this database confirms that 3% of individuals with RCC had a first-degree relative (FDR) with RCC.[19] Risk may be higher in individuals who have multiple FDRs with RCC and in women. Another study examined all patients in Iceland who developed RCC between 1955 and 1999 (1,078 cases). Investigators used an extensive computerized database to perform a unique genealogical study that included more than 600,000 Icelandic individuals. Results revealed that nearly 60% of Icelandic patients with RCC had a FDR or a second-degree relative (SDR) with RCC. Siblings of patients with RCC had an estimated RR of 2.5.[4] In a Korean study, individuals who had FDRs with RCC had a 2.29-fold increased risk of RCC. Risk did not appear to differ whether the FDR was a mother, father, or a sibling.[20] Modifying factors like obesity and hyperglycemia may further increase RCC risk in this high-risk population. Another study evaluated 80,309 monozygotic twins and 123,382 same-sex dizygotic twins in Denmark, Finland, Norway, and Sweden.[18] This study found excess cancer risk in twins whose co-twin was diagnosed with cancer. The estimated cumulative risks were an absolute 5% higher (95% confidence interval [CI], 4%–6%) in dizygotic twins (37%; 95% CI, 36%–38%) and an absolute 14% higher (95% CI, 12%–16%) in monozygotic twins (46%; 95% CI, 44%–48%)—for those whose co-twin also developed cancer—than in the overall cohort (32%). Overall heritability of cancer, calculated by assessing the relative contribution of heredity versus shared environment, was estimated to be 33%. Kidney cancer heritability was estimated to be 38% (95% CI, 21%–55%). Shared environmental factors did not significantly contribute to overall risk.

Young age at RCC onset is also a clue that hereditary etiology is possible. Unlike sporadic RCC, which is generally diagnosed during the fifth to seventh decades of life, hereditary forms of RCC are generally diagnosed at an earlier age. In a review of more than 600 cases of hereditary RCC from the National Cancer Institute, the median age of RCC diagnosis was 37 years, with 70% of cases being diagnosed at age 46 years or younger.[3] This age is lower than the median age of RCC diagnosis in the general population, which is 64 years.[21] Heritable RCCs are often multifocal and bilateral. A retrospective analysis of 1,235 patients with RCC who underwent genetic testing revealed that 6.1% of this population had positive genetic test results, 75.5% had negative test results, and 18.4% had a variant of unknown significance. Young age at RCC diagnosis was the only variable associated with a positive test result.[8] Other series showed that patients with non-clear cell advanced RCC may have an enrichment for pathogenic variants when compared with patients who had clear cell RCC; however, current research data are limited.[5,22]

While there is much debate about the referral criteria for hereditary RCC genetic testing, the following organizations have offered some guidance:

These guidelines acknowledge that the following criteria can prompt a referral to genetic counseling: early age of RCC onset, family history of RCC (≥1 FDR/SDR with RCC), bilateral or multifocal RCCs, and suspicious RCC histology. A consensus statement published by a group of kidney cancer experts provides additional guidance that may help providers identify patients who can be referred to genetic counseling.[25]

When evaluating patients at risk of hereditary kidney cancer, specific clinical features help determine which test is the most appropriate to order. Single gene tests are available during family variant testing or when there is only suspicion for one specific kidney cancer syndrome. The following panel tests are also available: 1) broad cancer genetic panels of up to 100 genes associated with cancer predisposition, and 2) renal cancer genetic panels with 15 to 20 genes that have strong associations with hereditary kidney cancer syndromes. Most of these panels conduct targeted sequencing of the exon with little coverage of the intron, except for splice-site variants. In the future, RNA testing may be useful to evaluate variants of unknown significance identified by DNA testing, to add additional support for pathogenicity. Whole genome sequencing (WGS) can be considered for rare cases with clinical suspicion that had negative panel testing. WGS may detect structural variants in introns that can contribute to cancer predisposition. In a series of over 1,300 unselected patients with RCC who underwent WGS, 6.9% of patients had germline pathogenic variants identified in cancer predisposition genes.[12]

Other Risk Factors for Renal Cell Carcinoma

Studies of environmental and lifestyle factors contributing to the risk of RCC focus almost exclusively on sporadic (i.e., nonhereditary) RCC. Smoking, hypertension, and obesity are the major environmental and lifestyle risk factors associated with RCC.[26] In addition, workers who were reportedly exposed to the environmental carcinogen trichloroethylene developed sporadic clear cell RCC, presumably resulting from somatic variants in the VHL gene.[27] Dietary intake of vegetables and fruits has been inversely associated with RCC. Greater intake of red meat and milk products have been associated with increased RCC risk, although not consistently.[28]

Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the genetics of renal cell carcinoma. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.

Reviewers and Updates

This summary is reviewed regularly and updated as necessary by the PDQ Cancer Genetics Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

Board members review recently published articles each month to determine whether an article should:

Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.

The lead reviewers for Genetics of Renal Cell Carcinoma are:

Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website’s Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

Levels of Evidence

Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Cancer Genetics Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.

Permission to Use This Summary

PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”

The preferred citation for this PDQ summary is:

PDQ® Cancer Genetics Editorial Board. PDQ Genetics of Renal Cell Carcinoma. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/kidney/hp/renal-cell-carcinoma-genetics. Accessed <MM/DD/YYYY>. [PMID: 26389510]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in Visuals Online, a collection of over 2,000 scientific images.

Disclaimer

The information in these summaries should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s Email Us.

Transitional cell cancer of the renal pelvis and ureter is a type of cancer that forms in the transitional cells of the renal pelvis and ureter.

Transitional cells are a type of cell that lines the inside of the renal pelvis, ureters, and other organs. These cells are able to stretch when the renal pelvis or bladder is full of urine and shrink when it is emptied. Transitional cell cancer can form in the renal pelvis, the ureters, or both.

The renal pelvis is the area at the center of the kidney. It collects urine before it flows into the ureters, which are long tubes that connect the kidney to the bladder. There are two kidneys, one on each side of the backbone, above the waist. The kidneys of an adult are about 5 inches long and 3 inches wide and are shaped like a kidney bean.

The kidneys play an important role in the urinary system:

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Renal cell cancer is a more common type of kidney cancer. For more information, see Renal Cell Cancer Treatment.

A personal history of bladder cancer and smoking can increase the risk of transitional cell cancer of the renal pelvis and ureter.

Transitional cell cancer of the renal pelvis and ureter is caused by certain changes to the way transitional cells in the renal pelvis and ureters function, especially how they grow and divide into new cells. There are many risk factors for transitional cell cancer of the renal pelvis and ureter, but many do not directly cause cancer. Instead, they increase the chance of DNA damage in cells that may lead to cancer. To learn more about how cancer develops, see What Is Cancer?

A risk factor is anything that increases the chance of getting a disease. Some risk factors for transitional cell cancer of the renal pelvis and ureter, like smoking, can be changed. However, risk factors may also include things you cannot change, like your personal medical history. Learning about risk factors for transitional cell cancer of the renal pelvis and ureter can help you make changes that might lower your risk of getting it.

Risk factors for transitional cell cancer of the renal pelvis and ureter include:

Having one or more of these risk factors does not mean that you will get transitional cell cancer of the renal pelvis and ureter. Many people with risk factors never develop this type of cancer, while others with no known risk factors do. Talk with your doctor if you think you might be at risk.

Signs and symptoms of transitional cell cancer of the renal pelvis and ureter include blood in the urine and back pain.

In the early stages, there may be no signs and symptoms of transitional cell cancer of the renal pelvis and ureter. Symptoms may appear as the tumor grows and may include:

These symptoms may be caused by transitional cell cancer of the renal pelvis and ureter or by other conditions. It’s important to check with your doctor if you have any of these symptoms to find out the cause and begin treatment if needed.

Tests that examine the abdomen and kidneys are used to diagnose transitional cell cancer of the renal pelvis and ureter.

In addition to asking about your personal and family health history and doing a physical exam, your doctor may perform the following tests and procedures:

After transitional cell cancer of the renal pelvis and ureter has been diagnosed, tests are done to find out if cancer cells have spread within the renal pelvis and ureter or to other parts of the body.

The process used to find out if the cancer has spread within the renal pelvis and ureter or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. The results of the diagnostic and staging tests will also help you and your doctor plan treatment.

The following tests and procedures may also be used in the staging process:

Some people decide to get a second opinion.

You may want to get a second opinion to confirm your cancer diagnosis and treatment plan. If you seek a second opinion, you will need to get medical test results and reports from the first doctor to share with the second doctor. The second doctor will review the pathology report, slides, and scans. They may agree with the first doctor, suggest changes or another treatment approach, or provide more information about your cancer.

To learn more about choosing a doctor and getting a second opinion, see Finding Cancer Care. You can contact NCI’s Cancer Information Service via chat, email, or phone (both in English and Spanish) for help finding a doctor, hospital, or getting a second opinion. For questions you might want to ask at your appointments, see Questions to Ask Your Doctor about Cancer.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis depends on the stage and grade of the tumor.

The treatment options depend on:

Most transitional cell cancer of the renal pelvis and ureter can be cured if found early.

Cancer stage describes the extent of cancer in the body, such as the size of the tumor, whether it has spread, and how far it has spread from where it first formed. It is important to know the stage of transitional cell cancer of the renal pelvis and ureter to plan the best treatment.  

There are several staging systems for cancer that describe the extent of the cancer. Transitional cell cancer of the renal pelvis and ureter staging usually uses the TNM staging system. You may see your cancer described by this staging system in your pathology report. Based on the TNM results, a stage (I, II, III, or IV, also written as 1, 2, 3, or 4) is assigned to your cancer. When talking to you about your cancer, your doctor may describe it as one of these stages. 

Learn more about Cancer Staging.

The following stages are used for transitional cell cancer of the renal pelvis and/or ureter:

Transitional cell cancer of the renal pelvis and ureter is also described as localized, regional, metastatic, or recurrent:

There are different types of treatment for patients with transitional cell cancer of the renal pelvis and ureter.

Different types of treatments are available for people with transitional cell cancer of the renal pelvis and ureter. You and your cancer care team will work together to decide your treatment plan, which may include more than one type of treatment. Many factors will be considered, such as the stage of the cancer, your overall health, and your preferences. Your plan will include information about your cancer, the goals of treatment, your treatment options and the possible side effects, and the expected length of treatment. 

Talking with your cancer care team before treatment begins about what to expect will be helpful. You’ll want to learn what you need to do before treatment begins, how you’ll feel while going through it, and what kind of help you will need. To learn more, see Questions to Ask Your Doctor about Treatment. 

The following type of treatment is used:

New types of treatment are being tested in clinical trials.

For some people, joining a clinical trial may be an option. There are different types of clinical trials for people with cancer. For example, a treatment trial tests new treatments or new ways of using current treatments. Supportive care and palliative care trials look at ways to improve quality of life, especially for those who have side effects from cancer and its treatment.

You can use the clinical trial search to find NCI-supported cancer clinical trials accepting participants. The search allows you to filter trials based on the type of cancer, your age, and where the trials are being done. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

Learn more about clinical trials, including how to find and join one, at Clinical Trials Information for Patients and Caregivers.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied.

Treatment for transitional cell cancer of the renal pelvis and ureter may cause side effects.

For information about side effects caused by treatment for cancer, visit our Side Effects page.

Follow-up care may be needed.

As you go through treatment, you will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back).

About PDQ

Physician Data Query (PDQ) is the National Cancer Institute’s (NCI’s) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.

PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of transitional cell cancer of the renal pelvis and ureter. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

Reviewers and Updates

Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary (“Updated”) is the date of the most recent change.

The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.

Clinical Trial Information

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become “standard.” Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Clinical trials can be found online at NCI’s website. For more information, call the Cancer Information Service (CIS), NCI’s contact center, at 1-800-4-CANCER (1-800-422-6237).

Permission to Use This Summary

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Transitional Cell Cancer of the Renal Pelvis and Ureter Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/kidney/patient/transitional-cell-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389285]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.

Incidence and Mortality

Transitional cell cancer of the renal pelvis accounts for only 7% of all kidney tumors, and transitional cell cancer of the ureter accounts for only 4% of upper urinary tract tumors. These cancers are curable in more than 90% of patients if they are superficial and confined to the renal pelvis or ureter. Patients with deeply invasive tumors that are confined to the renal pelvis or ureter have a 10% to 15% likelihood of cure. Patients with tumors with penetration through the urothelial wall or with distant metastases usually cannot be cured with available forms of treatment.

Prognosis

The major prognostic factor at the time of diagnosis of upper tract transitional cell cancer is the depth of infiltration into or through the uroepithelial wall.

Most superficial tumors are likely to be well differentiated, while infiltrative tumors are likely to be poorly differentiated. The incidence of synchronous or metachronous contralateral upper tract cancers ranges from 2% to 4%; the incidence of subsequent bladder cancer after previous upper tract transitional cell cancer ranges from 30% to 50%.[1] When involvement of the upper tract is diffuse (involving both the renal pelvis and ureter), the likelihood of subsequent bladder cancer increases to 75%. DNA ploidy has not added significant prognostic information beyond that provided by stage and grade.[2]

Diagnostics

Even if ureteroscopy and pyeloscopy are successfully implemented, accurate assessment of depth of invasion is difficult.

Treatment Management and Survivorship

Total excision of the ureter with a bladder cuff, renal pelvis, and kidney is recommended to provide the greatest likelihood of cure.

AJCC Stage Groupings and TNM Definitions

The American Joint Committee on Cancer (AJCC) has designated staging by TNM (tumor, node, metastasis) classification to define carcinoma of the renal pelvis and ureter.[6]

Patients may also be designated as having localized, regional, or metastatic disease, as follows:

Each of these classifications has been subclassified into categories of unicentricity or multicentricity. The latter category indicates a more pervasive tumor diathesis and generally a less favorable prognosis.

Although the classifications listed above have prognostic significance, they can be determined only at the time of nephroureterectomy, which is the treatment of choice for patients with this disease. Because of the high incidence of tumor recurrence within the intramural ureter among patients who have had incomplete excision of this area, nephroureterectomy includes the entire ureter and a margin of periureteral orifice mucosa (i.e., bladder cuff).

A TNM staging system has demonstrated accurate predictions of survival. The TNM staging system may be a better predictor of prognosis than tumor grade, although both are strongly predictive of survival. Median survival for patients with tumors confined to the subepithelial connective tissue was 91.1 months, compared with 12.9 months for patients with tumors invading the muscularis and beyond, in one report. Flow cytometry analysis identifies low-stage, low-grade tumors at high risk of recurrence by virtue of their aneuploid histograms.[7,8]

Treatment Options for Localized Transitional Cell Cancer of the Renal Pelvis and Ureter

Treatment options for localized transitional cell cancer of the renal pelvis and ureter include the following:

The development of new instrumentation for endourological treatment of upper tract transitional cell cancer has provided new options for regional management of these cancers. Introduction of electrofulguration and resection instruments or laser probes either transureterally or percutaneously, may permit destruction of a primary cancer. Introduction of cytotoxic agents has also been used. Although a biopsy can be taken for staging purposes, its accuracy remains to be determined. The efficacy of treatment by these maneuvers has not been established.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of transitional cell cancer of the renal pelvis and ureter. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.

Reviewers and Updates

This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

Board members review recently published articles each month to determine whether an article should:

Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.

The lead reviewer for Transitional Cell Cancer of the Renal Pelvis and Ureter Treatment is:

Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website’s Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

Levels of Evidence

Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.

Permission to Use This Summary

PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”

The preferred citation for this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Transitional Cell Cancer of the Renal Pelvis and Ureter Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/kidney/hp/transitional-cell-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389446]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in Visuals Online, a collection of over 2,000 scientific images.

Disclaimer

Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s Email Us.

Renal cell cancer is a type of cancer that forms in tubules of the kidney.

Renal cell cancer (also called renal cell adenocarcinoma) is a disease in which cancer cells are found in the lining of tubules (very small tubes) in the kidney. It is the most common type of kidney cancer in adults. There are two kidneys, one on each side of the backbone, above the waist. Tiny tubules in the kidneys filter and clean the blood. They take out waste products and make urine. The urine passes from each kidney through a long tube called a ureter into the bladder. The bladder holds the urine until it passes through the urethra and leaves the body.

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Cancer that starts in the ureters or the renal pelvis (the part of the kidney that collects urine and drains it to the ureters) is different from renal cell cancer. For more information, visit Transitional Cell Cancer of the Renal Pelvis and Ureter Treatment.

Smoking and misuse of certain pain medicines can affect the risk of renal cell cancer.

Renal cell cancer is caused by certain changes to the way renal cells function, especially how they grow and divide into new cells. There are many risk factors for renal cell cancer, but many do not directly cause cancer. Instead, they increase the chance of DNA damage in cells that may lead to renal cell cancer. Learn more about how cancer develops at What Is Cancer?

A risk factor is anything that increases the chance of getting a disease. Some risk factors for renal cell cancer, like smoking, can be changed. However, risk factors also include things you cannot change, like your genetics, getting older, and your family history. Learning about risk factors for renal cell cancer can help you make changes that might lower your risk of getting it.

Risk factors for renal cell cancer include:

Tobacco use is a leading cause of cancer and death from cancer. Learn more about Tobacco (includes help with quitting).

Having one or more of these risk factors does not mean you will get renal cell cancer. Many people with risk factors never develop renal cell cancer, while others with no known risk factors do. Talk with your doctor if you think you may be at risk.

Signs of renal cell cancer include blood in the urine and a lump in the abdomen.

These and other signs and symptoms may be caused by renal cell cancer or by other conditions. There may be no signs or symptoms in the early stages. Signs and symptoms may appear as the tumor grows. Check with your doctor if you have:

Tests that examine the abdomen and kidneys are used to diagnose renal cell cancer.

In addition to asking about your personal and family health history and doing a physical exam, your doctor may perform the following tests and procedures:

After renal cell cancer has been diagnosed, tests are done to find out if cancer cells have spread within the kidney or to other parts of the body.

The process used to find out if cancer has spread within the kidney or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment.

Some of the tests and procedures used to diagnose liver cancer, such as CT scan and MRI, may be used in the staging process. Other tests include:

Some people decide to get a second opinion.

You may want to get a second opinion to confirm your renal cell cancer diagnosis and treatment plan. If you seek a second opinion, you will need to get medical test results and reports from the first doctor to share with the second doctor. The second doctor will review the pathology report, slides, and scans. They may agree with the first doctor, suggest changes or another treatment approach, or provide more information about your cancer.

To learn more about choosing a doctor and getting a second opinion, see Finding Cancer Care. You can contact NCI’s Cancer Information Service via chat, email, or phone (both in English and Spanish) for help finding a doctor, hospital, or getting a second opinion. For questions you might want to ask at your appointments, see Questions to Ask Your Doctor About Cancer.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis and treatment options depend on:

Cancer stage describes the extent of cancer in the body, such as the size of the tumor, whether it has spread, and how far it has spread from where it first formed.

There are several staging systems for cancer that describe the extent of the cancer. Renal cell cancer staging usually uses the TNM staging system. The cancer may be described by this staging system in your pathology report. Based on the TNM results, a stage (I, II, III, or IV, also written as 1, 2, 3, or 4) is assigned to your cancer. When talking to you about your diagnosis, your doctor may describe the cancer as one of these stages.

Learn about tests to stage renal cell cancer. Learn more about Cancer Staging.

The following stages are used for renal cell cancer:

Stage I (also called stage 1) renal cell cancer

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In stage I, the tumor is 7 centimeters or smaller and is found in the kidney only.

Stage II (also called stage 2) renal cell cancer

Enlarge

In stage II, the tumor is larger than 7 centimeters and is found in the kidney only.

Stage III (also called stage 3) renal cell cancer

Enlarge

In stage III, one of the following is found:

• the cancer in the kidney is any size, and cancer has spread to nearby lymph nodes; or
• cancer has spread to blood vessels in or near the kidney (renal vein or vena cava), to the fat around the structures in the kidney that collect urine, or to the layer of fatty tissue around the kidney and may have spread to nearby lymph nodes.

Stage IV (also called stage 4) renal cell cancer

Enlarge

In stage IV, one of the following is found:

• cancer has spread beyond the layer of fatty tissue around the kidney and may have spread into the adrenal gland above the kidney with cancer or to nearby lymph nodes; or
• cancer has spread to other parts of the body, such as the bones, liver, lungs, brain, adrenal glands, or distant lymph nodes.

Stage IV renal cell cancer is also called metastatic renal cell cancer. Metastatic cancer happens when cancer cells travel through the lymphatic system or blood and form tumors in other parts of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if renal cell cancer spreads to the liver, the cancer cells in the liver are actually renal cell cancer. The disease is called metastatic renal cell cancer, not liver cancer. Learn more in Metastatic Cancer: When Cancer Spreads.

Renal cell cancer can recur (come back) many years after initial treatment.

Recurrent renal cell cancer is cancer that has come back after it has been treated. If renal cell cancer comes back, it may come back in the kidney or in other parts of the body. Tests will be done to help determine where the cancer has returned. The type of treatment for recurrent renal cell cancer will depend on where it has come back.

Learn more in Recurrent Cancer: When Cancer Comes Back.

There are different types of treatment for people with renal cell cancer.

Different types of treatments are available for people with renal cell cancer. You and your cancer care team will work together to decide your treatment plan, which may include more than one type of treatment. Many factors will be considered, such as the stage of the cancer, your overall health, and your preferences. Your plan will include information about your cancer, the goals of treatment, your treatment options and the possible side effects, and the expected length of treatment.

Talking with your cancer care team before treatment begins about what to expect will be helpful. You’ll want to learn what you need to do before treatment begins, how you’ll feel while going through it, and what kind of help you will need. To learn more, see Questions to Ask Your Doctor About Treatment.

The following types of treatment are used:

New types of treatment are being tested in clinical trials.

For some people, joining a clinical trial may be an option. There are different types of clinical trials for people with cancer. For example, a treatment trial tests new treatments or new ways of using current treatments. Supportive care and palliative care trials look at ways to improve quality of life, especially for those who have side effects from cancer and its treatment.

You can use the clinical trial search to find NCI-supported cancer clinical trials accepting participants. The search allows you to filter trials based on the type of cancer, your age, and where the trials are being done. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

Learn more about clinical trials, including how to find and join one, at Clinical Trials Information for Patients and Caregivers.

Treatment for renal cell cancer may cause side effects.

For information about side effects caused by treatment for cancer, visit our Side Effects page.

Follow-up care may be needed.

As you go through treatment, you will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back).

After treatment for renal cell cancer, a blood test to measure amounts of carcinoembryonic antigen (a substance in the blood that may be increased when cancer is present) may be done to see if the cancer has come back.

About PDQ

Physician Data Query (PDQ) is the National Cancer Institute’s (NCI’s) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.

PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of renal cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

Reviewers and Updates

Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary (“Updated”) is the date of the most recent change.

The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.

Clinical Trial Information

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become “standard.” Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Clinical trials can be found online at NCI’s website. For more information, call the Cancer Information Service (CIS), NCI’s contact center, at 1-800-4-CANCER (1-800-422-6237).

Permission to Use This Summary

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Renal Cell Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/kidney/patient/kidney-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389448]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.

Incidence and Mortality

Estimated new cases and deaths from renal cell (kidney and renal pelvis) cancer in the United States in 2025:[1]

Follow-Up and Survivorship

Renal cell cancer, also called renal adenocarcinoma or hypernephroma, can often be cured if it is diagnosed and treated when still localized to the kidney and the immediately surrounding tissue. The probability of cure is directly related to the stage or degree of tumor dissemination. Even when regional lymphatics or blood vessels are involved with the tumor, a significant number of patients can achieve prolonged survival and probable cure.[2] When distant metastases are present, disease-free survival is poor; however, some patients survive after surgical resection of all known tumor. Because most patients are diagnosed when the tumor is still relatively localized and amenable to surgical removal, approximately 75% of all patients with renal cell cancer survive for 5 years.[3] Occasionally, patients with locally advanced or metastatic disease may exhibit indolent courses lasting several years. Late tumor recurrence many years after initial treatment also occasionally occurs.

Renal cell cancer is one of the few tumors in which well-documented cases of spontaneous tumor regression in the absence of therapy exist, but this regression occurs rarely and may not lead to long-term survival.

Treatment Modalities

Surgical resection is the mainstay of treatment of this disease. Even in patients with disseminated tumor, locoregional forms of therapy may play an important role in palliating symptoms of the primary tumor or of ectopic hormone production. Systemic therapy has demonstrated only limited effectiveness.

AJCC Stage Groupings and TNM Definitions

The American Joint Committee on Cancer (AJCC) has designated staging by TNM (tumor, node, metastasis) classification to define renal cell cancer.[5]

Table 1. Definitions of TNM Stage Ia

Stage	TNM	Definition	Illustration
T = primary tumor; N = regional lymph node; M = distant metastasis.
aReprinted with permission from AJCC: Kidney. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 739–48.
I	T1, N0, M0	T1 = Tumor ≤7 cm in greatest dimension, limited to the kidney.	Enlarge
		–T1a = Tumor ≤4 cm in greatest dimension, limited to the kidney.
		–T1b = Tumor >4 cm but ≤7 cm in greatest dimension, limited to the kidney.
		N0 = No regional lymph node metastasis.
		M0 = No distant metastasis.

Table 2. Definitions of TNM Stage IIa

Stage	TNM	Definition	Illustration
T = primary tumor; N = regional lymph node; M = distant metastasis.
aReprinted with permission from AJCC: Kidney. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 739–48.
II	T2, N0, M0	T2 = Tumor >7 cm in greatest dimension, limited to the kidney.	Enlarge
		–T2a = Tumor >7 cm but ≤10 cm in greatest dimension, limited to the kidney.
		–T2b = Tumor >10 cm, limited to the kidney.
		N0 = No regional lymph node metastasis.
		M0 = No distant metastasis.

Table 3. Definitions of TNM Stage IIIa

Stage	TNM	Definition	Illustration
T = primary tumor; N = regional lymph node; M = distant metastasis.
aReprinted with permission from AJCC: Kidney. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 739–48.
III	T1, N1, M0	T1 = Tumor ≤7 cm in greatest dimension, limited to the kidney.	Enlarge
		–T1a = Tumor ≤4 cm in greatest dimension, limited to the kidney.
		–T1b = Tumor >4 cm but ≤7 cm in greatest dimension, limited to the kidney.
		N1 = Metastasis in regional lymph node(s).
		M0 = No distant metastasis.
	T2, N1, M0	T2 = Tumor >7 cm in greatest dimension, limited to the kidney.
		–T2a = Tumor >7 cm but ≤10 cm in greatest dimension, limited to the kidney.
		–T2b = Tumor >10 cm, limited to the kidney.
		N1 = Metastasis in regional lymph node(s).
		M0 = No distant metastasis.
	T3, N0, M0	T3 = Tumor extends into major veins or perinephric tissues but not into the ipsilateral adrenal gland and not beyond Gerota’s fascia.
		–T3a = Tumor extends into the renal vein or its segmental branches, or invades the pelvicalyceal system, or invades perirenal and/or renal sinus fat but not beyond Gerota’s fascia.
		–T3b = Tumor extends into the vena cava below the diaphragm.
		–T3c = Tumor extends into the vena cava above the diaphragm or invades the wall of the vena cava.
		N0 = No regional lymph node metastasis.
		M0 = No distant metastasis.
	T3, N1, M0	T3 = Tumor extends into major veins or perinephric tissues but not into the ipsilateral adrenal gland and not beyond Gerota’s fascia.
		–T3a = Tumor extends into the renal vein or its segmental branches, or invades the pelvicalyceal system, or invades perirenal and/or renal sinus fat but not beyond Gerota’s fascia.
		–T3b = Tumor extends into the vena cava below the diaphragm.
		–T3c = Tumor extends into the vena cava above the diaphragm or invades the wall of the vena cava.
		N1 = Metastasis in regional lymph node(s).
		M0 = No distant metastasis.

Table 4. Definitions of TNM Stage IVa

Stage	TNM	Definition	Illustration
T = primary tumor; N = regional lymph node; M = distant metastasis.
aReprinted with permission from AJCC: Kidney. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 739–48.
IV	T4, Any N, M0	T4 = Tumor invades beyond Gerota’s fascia (including contiguous extension into the ipsilateral adrenal gland).	Enlarge
		NX = Regional lymph nodes cannot be assessed.
		N0 = No regional lymph node metastasis.
		N1 = Metastasis in regional lymph node(s).
		M0 = No distant metastasis.
	Any T, Any N, M1	TX = Primary tumor cannot be assessed.
		T0 = No evidence of primary tumor.
		T1 = Tumor ≤7 cm in greatest dimension, limited to the kidney.
		–T1a = Tumor ≤4 cm in greatest dimension, limited to the kidney.
		–T1b = Tumor >4 cm but ≤7 cm in greatest dimension, limited to the kidney.
		T2 = Tumor >7 cm in greatest dimension, limited to the kidney.
		–T2a = Tumor >7 cm but ≤10 cm in greatest dimension, limited to the kidney.
		–T2b = Tumor >10 cm, limited to the kidney.
		T3 = Tumor extends into major veins or perinephric tissues but not into the ipsilateral adrenal gland and not beyond Gerota’s fascia.
		–T3a = Tumor extends into the renal vein or its segmental branches, or invades the pelvicalyceal system, or invades perirenal and/or renal sinus fat but not beyond Gerota’s fascia.
		–T3b = Tumor extends into the vena cava below the diaphragm.
		–T3c = Tumor extends into the vena cava above the diaphragm or invades the wall of the vena cava.
		T4 = Tumor invades beyond Gerota’s fascia (including contiguous extension into the ipsilateral adrenal gland).
		NX = Regional lymph nodes cannot be assessed.
		N0 = No regional lymph node metastasis.
		N1 = Metastasis in regional lymph node(s).
		M1 = Distant metastasis.

Treatment Options for Stage I Renal Cell Cancer

Treatment options for stage I renal cell cancer include:

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Treatment Options for Stage II Renal Cell Cancer

Treatment options for stage II renal cell cancer include:

Surgical resection is the accepted, often curative, therapy for patients with stage II renal cell cancer. In appropriately selected patients, partial nephrectomy has oncologic outcomes that are comparable with those of radical nephrectomy. Partial nephrectomy also has the benefits of preserving greater renal function and, according to some studies, reduced mortality.[3–6] When the entire kidney is removed, resection may be simple or radical. Radical resection includes removal of the kidney, adrenal gland, perirenal fat, and Gerota’s fascia, with or without a regional lymph node dissection. Radical nephrectomy is preferred if the tumor extends into the inferior vena cava. Lymphadenectomy is commonly used, but its effectiveness has not been definitively proven.

Postoperative systemic therapy with the anti-programmed death-1 (PD-1) antibody pembrolizumab has been shown to prolong disease-free survival (DFS), but not overall survival (OS), in patients with high-risk pT2 tumors.

In patients who are not candidates for surgery, arterial embolization can provide palliation.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Treatment Options for Stage III Renal Cell Cancer

Treatment options for stage III renal cell cancer include:

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Treatment Options for First-Line Therapy for Stage IV Renal Cell Cancer

Treatment options for first-line therapy for stage IV renal cell cancer include:

Treatment Options for Second-Line Therapy for Stage IV Renal Cell Cancer

Treatment options for second-line therapy for stage IV renal cell cancer include:

Treatment Options for Third- and Fourth-Line Therapy for Stage IV Renal Cell Cancer

Treatment options for third- and fourth-line therapy for stage IV renal cell cancer include:

The prognosis is poor for any patient with renal cell cancer that is progressing, recurring, or relapsing after treatment, regardless of cancer cell type or stage of disease. Almost all patients with stage IV renal cell cancer have incurable disease. The use and selection of further treatment depends on many factors, including previous treatment and site of recurrence, as well as individual patient considerations. Carefully selected patients may benefit from surgical resection of localized metastatic disease, particularly if they have had a prolonged disease-free interval since their primary therapy.

Immunotherapy

Combined Immune Checkpoint Inhibitors and Antiangiogenic Targeted Therapies

After immune checkpoint inhibitors and antiangiogenic targeted therapies were found to improve outcomes, clinical trials of the combination of these two approaches showed longer OS when compared with monotherapy.

Local Therapy

In patients without metastatic disease, resection of the primary tumor, when feasible, is standard practice. In patients with unresectable and/or metastatic cancers, tumor embolization, EBRT, and nephrectomy can aid in the palliation of symptoms caused by the primary tumor or related ectopic hormone or cytokine production.

Antiangiogenic and Other Targeted Therapy

A growing understanding of the biology of cancer in general, and renal cell cancer in particular, has led to the development and U.S. Food and Drug Administration (FDA) approval of six new agents that target specific growth pathways. Two of the approved targeted therapies block the mammalian target of rapamycin (mTOR), a serine/threonine protein kinase that regulates cell growth, division, and survival.

Combined Therapy With Multitargeted TKIs and mTOR Inhibitors

Chemotherapy

Responses to cytotoxic chemotherapy generally have not exceeded 10% for any regimen that has been studied in adequate numbers of patients.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of renal cancer. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.

Reviewers and Updates

This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

Board members review recently published articles each month to determine whether an article should:

Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.

The lead reviewers for Renal Cell Cancer Treatment are:

Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website’s Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

Levels of Evidence

Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.

Permission to Use This Summary

PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”

The preferred citation for this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Renal Cell Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/kidney/hp/kidney-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389256]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in Visuals Online, a collection of over 2,000 scientific images.

Disclaimer

Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s Email Us.