Receiving a kidney cancer diagnosis can be overwhelming, but you are not alone. Approximately 74,000 new cases of kidney cancer were expected to be diagnosed in the United States in 2019. Globally, kidney cancer is the ninth most common cancer among men and the 14th most common cancer among women.
Today, kidney cancer is a major focus of cancer research with new findings published and new drug approvals by the FDA on an ongoing basis. Significant research advances have helped us understand more about what causes kidney cancer as well as how best to detect and treat it.
Although people with a kidney cancer diagnosis share many similarities, there are many differences between kidney cancers including different types and different treatments.
The ‘Patients’ section can help you learn more about kidney cancer including known causes, possible symptoms, how a diagnosis is made, how kidney cancer is staged, and the different treatment options and side effects. You can also find information about clinical trials and resources for living with kidney cancer.
Kidney cancer is commonly referred to as renal cell carcinoma (RCC). There are various forms of RCC including clear cell, papillary, sarcomatoid, and transitional cell. The type of treatment prescribed is based on the type of kidney cancer, stage, and grade of the tumor. The stage describes the size and extent of spread of the original tumor. An early stage cancer is a tumor localized to the kidney while a later stage cancer has spread or metastasized to other areas. The grade* of the tumor describes how abnormal the tumor’s cell appear under a microscope and can be an indicator of how quickly a tumor is likely to grow.
Treatment options may include surgery to remove the tumor, systemic treatment (treatment taken by mouth or given by an intravenous infusion), or radiation therapy.
Understanding the stage of your cancer and the potential treatment options can help you have more informed discussions with your healthcare team and increase your confidence that you are making the right decision about your health and treatment.
*The Fuhrman grading scale indicates how much cancer cells appear to be normal (grade 1) to increasingly abnormal (grades 2-4).
Grade 1: Tumor cell nuclei are round and uniform.
Grade 2: Nuclei are slightly irregular.
Grade 3: Nuclei are markedly irregular.
Grade 4: Nuclei are large and very irregular or multilobular.
Not all kidney cancers are the same and there are several different subtypes, or cell types. A specially trained pathologist determines the type of kidney cancer by examining a sample of tissue under a microscope. The treatment plan is partly based on the pathology report, which identifies the kidney cancer subtype. Your healthcare team can help you understand your tumor type and pathology – these help to determine treatment options will be best for you.
This is the most common form of kidney cancer and represents between 66% and 75% of all cases. Clear cell RCC is the cell type associated with a mutation or alteration in the von Hippel-Lindau (VHL) gene. Mutations in the VHL gene are associated with approximately 70% of non-inherited clear cell type kidney cancers. Clear cell type that is localized is typically treated with surgery and clear-cell type that has spread or metastasized is usually treated with systemic treatment.
Because clear cell RCC is the most common type of kidney cancer, it is the most frequently studied kidney cancer subtype. In the last 15 years, many drugs have received FDA approval for treating metastatic clear cell RCC.
This is the second most common form of kidney cancer, making up approximately 15% of RCC cases. There are two main types of papillary RCC – type 1 and type 2. Type 1 tumors tend to be slower growing and cured with surgery whereas type 2 tend to be faster growing and more likely to spread or metastasize. Papillary RCC may be heredity (run in families) or non-hereditary (sporadic).
Depending on the type and stage of papillary type RCC, the treatments typically prescribed are surgery or systemic therapy. There is research being done to better understand papillary type RCC and to find the best treatment options.
This is a rare form of kidney cancer that comprises approximately 5% of all RCC. Chromophone type RCC originates in the cells that line the small tubules in the kidney, the parts that help to filters the waste from blood, making urine. Chromophobe type RCC may be heredity or non-hereditary. The usual recommended treatment in surgery.
This is a benign tumor of the kidney that makes up approximately 5% of all kidney tumors. These tumors do not metastasize, although they can grow to a large size in the kidney and invade local structures, which can result in symptoms requiring surgery. They are thought to be related to chromophobe type RCC, and it can be quite difficult to differentiate the two. The tumor is treated by a partial or complete surgical removal of the kidney.
Less than 1% of RCCs are an unclassified type – they are very rare and do not easily fit into one of the more common subtypes of RCC. When examined under a microscope, these unclassified cancer cells have a structure and genetic features that don’t match the description of the more common RCC subtypes. This category usually includes aggressive tumors that do not respond to traditional RCC therapy.
This is a rare and very aggressive type of RCC that represents less than 1% of cases. This form of RCC is usually metastatic at the time of diagnosis and is more common in younger individuals. Treatment has been directed at using systemic-based regimens similar to those used in the treatment of transitional cell carcinoma, as these tumors do not respond to traditional RCC therapies such as targeted therapy and immunotherapy.
This condition, known as “differentiation,” can occur with any of the common RCC subtypes in various percentages. The term refers to the fact that the RCC cells when viewed under the microscope have the appearance of sarcoma-like cells. and relates to the tumor’s aggressiveness. The prognosis associated with purely sarcomatoid RCC is usually poor. The condition is found frequently in patients whose kidney cancer has metastasized widely. Sarcomatoid RCC is frequently treated with systemic therapy.
Transitional cell carcinoma (TCC) of the kidney is a rare and potentially very aggressive tumor that should not be considered a true kidney cancer, but instead should be grouped with those cancers that develop from cells that line the urinary tract. This includes TCC of the urinary bladder, which is far more common than TCC of the kidney. If the cancer has not spread, the tumor can be treated by surgical removal of both the kidney and its ureter, although recurrences of TCC in the bladder are common. When the tumor is large or has metastasized, the prognosis is extremely poor, and treatment options are like those for metastatic urinary bladder cancer, which includes chemotherapy.
Translocation RCC is a rare type of kidney cancer characterized by alterations in the TFE3 gene. This type of RCC occurs predominantly in children and young adults but can also occur in older adults. For tumors that are localized, surgery is prescribed. For tumors that have spread or metastasized, the systemic therapies used to treat clear cell type RCC may be offered.
Renal medullary carcinoma (RMC) is an extremely rare variant of kidney cancer that affects young patients who carry the sickle cell trait or, more rarely, have sickle cell disease. Most commonly, these cancers have spread or are metastatic at the time of diagnosis. Depending on whether the tumor is localized or has spread, prescribed treatments may include surgery or systemic therapy. Because of the rarity of these tumors, uniform treatment guidelines have not yet been established. Contact the KCA’s Nurse Line for further information and an appropriate referral in your area.
Genetic factors have been identified and linked to an increased risk of developing kidney cancer. Understanding the genetic factors that may be involved in your cancer may also help guide treatment decisions. Your healthcare team will assess for genetic risk factors by asking you about your family history and may also recommend genetic testing. Below are common genetic syndromes that are linked to a risk of developing kidney cancer.
Von Hippel-Lindau (VHL) – VHL syndrome is a hereditary disorder that is associated with a high risk of developing kidney cancer. Scientists have isolated the gene responsible for VHL disease. This discovery has led to many important treatments and has increased our knowledge about the biology of kidney cancer for patients.
Birt-Hogg-Dubé (BHD) – BHD syndrome is a genetic disorder associated with kidney cancer that is characterized by the presence of multiple small bumps (nodules) on the skin covering the nose, cheeks, forehead, ears, and neck.
Hereditary leiomyomatosis and renal cell cancer (HLRCC) – HLRCC is a rare genetic disorder in which benign tumors grow on the skin. Women may also develop benign tumors (fibroids) in the smooth muscle tissue of the uterus. About 10-16 percent of people with HLRCC go on to develop kidney cancer, typically in their forties.
Hereditary papillary renal carcinoma (HPRC) – HPRC is a syndrome that can manifest as kidney cancer, typically in someone’s early 40s, though this can vary. HPRC may cause small tumors in the kidneys and/or larger lesions that result in symptoms like flank pain or blood in the urine. HPRC usually causes bilateral and multifocal type 1 papillary renal cell carcinoma.
Tuberous sclerosis – In some rare cases, tuberous sclerosis has been associated with renal cell carcinoma and there is a gene thought to be responsible for tuberous sclerosis. Tuberous sclerosis is a disease characterized by small tumors of the blood vessels that result in numerous bumps on the skin, mental retardation, seizures, and cysts in the kidneys, liver, and pancreas.
Some external factors such as smoking, obesity, uncontrolled high blood pressure, certain medications, and exposure to certain chemicals have been linked to a higher incidence of kidney cancer.