What Are the 4 Main Types of Kidney Cancer?

The four main types of kidney cancer are clear cell renal cell carcinoma, papillary renal cell carcinoma, chromophobe renal cell carcinoma, and collecting duct carcinoma. All four fall under the umbrella of renal cell carcinoma (RCC), which accounts for the vast majority of kidney cancers. They differ in how common they are, how fast they grow, and how they respond to treatment. A fifth type, transitional cell carcinoma of the renal pelvis, originates from a completely different cell type and behaves more like bladder cancer than traditional kidney cancer.

Clear Cell Renal Cell Carcinoma

Clear cell RCC is by far the most common form, making up roughly 70 to 80 percent of all renal cell carcinoma diagnoses. Its name comes from the way the cancer cells look under a microscope: the cell contents wash out during lab preparation, leaving them with a pale, “clear” appearance.

The genetic driver behind most clear cell tumors is a change in the VHL gene on chromosome 3. When this gene stops working properly, it disrupts the body’s ability to regulate blood vessel growth and oxygen sensing. People with the inherited condition Von Hippel-Lindau disease carry a faulty copy of this gene from birth and often develop multiple kidney tumors. In people without the inherited condition, the same gene can be damaged over a lifetime by environmental factors. Exposure to the industrial solvent trichloroethylene, for example, has been linked to VHL mutations and an increased risk of clear cell RCC.

Because clear cell RCC is so common, it has the most treatment options and clinical trial data behind it. For cancers caught before they spread, the five-year survival rate for kidney cancer overall is 93 percent. Once the disease reaches nearby tissues, that drops to about 76 percent, and for cancer that has spread to distant organs, the five-year survival rate falls to around 19 percent. For metastatic clear cell disease specifically, first-line treatment now typically involves immunotherapy combinations or immunotherapy paired with a targeted drug that blocks blood vessel growth. Several of these combinations have shown improvements in overall survival compared to older single-drug approaches.

Papillary Renal Cell Carcinoma

Papillary RCC is the second most common subtype, accounting for 13 to 20 percent of kidney tumors. It gets its name from finger-like projections (papillae) visible under the microscope. There are two distinct subtypes that matter for prognosis.

Type 1 papillary RCC is more common and tends to grow slowly. Type 2 is more aggressive, grows faster, and generally carries a worse outlook. This distinction matters because a diagnosis of papillary RCC alone doesn’t tell the full story. Your pathology report should specify which type you have.

Treatment for papillary RCC has historically lagged behind clear cell because the disease is less common and was often lumped into clinical trials alongside other subtypes. That’s changed in recent years. A targeted drug that blocks a protein called MET, which papillary tumors frequently rely on, showed nearly double the time before disease progression compared to an older standard therapy in a head-to-head trial (9 months versus 5.6 months). Newer immunotherapy combinations have also shown promising response rates in papillary RCC specifically, with some combinations producing tumor shrinkage in roughly half of patients.

Chromophobe Renal Cell Carcinoma

Chromophobe RCC accounts for about 5 percent of kidney cancers. Under the microscope, its cells are distinctive: large with prominent borders and wrinkled, irregular nuclei sometimes described as “raisin-like.” A variant with pink, granular cell contents and a characteristic halo around the nucleus also falls into this category.

The good news is that chromophobe RCC generally carries a better prognosis than clear cell RCC. Most studies show significantly higher five-year survival rates, though exact numbers vary depending on the stage at diagnosis. This subtype tends to grow more slowly and is less likely to spread to distant organs.

One diagnostic challenge is distinguishing chromophobe RCC from a benign tumor called an oncocytoma, which can look similar on imaging and even under the microscope. This distinction is critical because oncocytomas don’t require the same aggressive treatment. Your pathologist will use specialized staining techniques and sometimes genetic testing to tell the two apart. When chromophobe RCC does become metastatic, immunotherapy combinations have shown more modest response rates compared to clear cell disease, with about 28 percent of patients seeing tumor shrinkage in one recent study.

Collecting Duct Carcinoma

Collecting duct carcinoma is rare, representing less than 1 percent of all kidney cancers, and it is the most aggressive of the four types. It arises from cells deep in the kidney’s drainage system, in structures called the collecting ducts of Bellini, located in the inner part of the kidney (the medulla).

This cancer is disproportionately diagnosed in men, at roughly twice the rate of women, with a median age in the mid-50s, though cases have been reported in patients as young as 8 and as old as 83. Collecting duct carcinoma often presents at an advanced stage because tumors grow in the central part of the kidney and can become quite large before causing noticeable symptoms. By the time of diagnosis, the cancer has commonly invaded nearby blood vessels and lymph nodes.

Under the microscope, these tumors look infiltrative and poorly defined, with an inflammatory reaction surrounding them. The aggressive biology of collecting duct carcinoma means it responds poorly to many of the targeted therapies that work for clear cell RCC. Treatment approaches have historically borrowed from bladder cancer protocols because of some biological similarities, though newer immunotherapy combinations are being studied.

Transitional Cell Carcinoma of the Renal Pelvis

While not one of the four RCC subtypes, transitional cell carcinoma of the renal pelvis is worth knowing about because it starts in the kidney but behaves very differently. This cancer arises from the stretchy lining cells (transitional cells) that coat the hollow interior of the kidney where urine collects before flowing into the ureter. These same cells line the ureters and bladder, which is why this cancer is treated more like bladder cancer than kidney cancer.

The most common early sign is blood in the urine. Diagnosis typically involves urine tests to look for abnormal cells, imaging with CT or MRI, and often a procedure called ureteroscopy, where a thin scope is passed up through the ureter to directly visualize the renal pelvis and take tissue samples.

How Kidney Cancer Is Usually Found

Many kidney cancers produce no symptoms in their early stages. A large percentage are discovered incidentally during imaging done for unrelated reasons, like an abdominal CT scan for back pain. When symptoms do appear, the classic triad is blood in the urine, flank pain, and a palpable mass, though all three together are uncommon and usually indicate advanced disease.

Kidney cancer can also trigger systemic effects that seem unrelated to the kidneys. Anemia is the most common, affecting about 22 percent of patients. Other effects include unexplained fevers, high blood calcium levels, an overproduction of red blood cells, and significant weight loss. These occur because kidney tumors, particularly clear cell RCC, can secrete hormones and growth factors that affect distant parts of the body.

When a suspicious mass is found on imaging, radiologists use a classification system to assess kidney cysts and solid masses. Cysts are graded based on wall thickness, internal structures, and the presence of enhancing nodules. Thin-walled, simple cysts are almost always benign. Cysts with thick walls over 4 mm, irregular internal projections, or solid nodules carry a higher risk of malignancy and typically require surgical removal or close monitoring. Male sex and certain cyst features have been identified as independent risk factors for a cyst turning out to be cancerous.