Kidney cancer isn’t just one disease — it comes in different types and subtypes. One subtype, papillary renal cell carcinoma (PRCC), behaves differently from some of the more common forms, like clear cell renal cell carcinoma.

In this article, we’ll explain six key facts about PRCC, including what it is, who it’s most likely to affect, and how it’s treated. Becoming informed can help you feel more prepared for conversations with your healthcare team about treatment, symptoms, and your prognosis (outlook).

1. Papillary Renal Cell Carcinoma Is a Type of Kidney Cancer

Although papillary renal cell carcinoma is the second most common form of kidney cancer, it’s still quite rare. PRCC starts in the tubules — small structures in the kidney that help filter waste and extra fluid from the blood to form urine. PRCC is a form of renal cell carcinoma (RCC), the most common type of kidney cancer in adults. However, PRCC makes up only about 10 percent to 15 percent of all RCC cases.

Because PRCC is less common, fewer studies are available to help doctors guide treatment. This can make diagnosis and treatment choices feel more uncertain than with other types, like clear cell RCC, but effective options are available.

PRCC is often found by chance during tests for other medical issues. Many people don’t have symptoms early on. If you’ve been diagnosed, it’s important to talk with your healthcare provider. They can help you understand your next steps and cancer treatment options.

2. There Are Multiple Types of Papillary Renal Cell Carcinoma

Doctors used to group PRCC into just two types — types 1 and 2 — based on how the cancer cells look under a microscope. Researchers now know that PRCC is more complex. Today, doctors look closely at small changes in the tumor’s molecular components, like changes in genes or cell receptors, to better understand how the cancer might behave.

Still, you may hear your doctor refer to PRCC as type 1 or type 2. Here’s a quick overview of each.

Type 1 PRCC usually grows slowly and tends to be less aggressive. Because of this, type 1 is often easier to treat and may have a better prognosis than type 2. Some cases of type 1 are linked to inherited conditions, such as hereditary papillary renal cancer, which can run in families. People with this condition may develop tumors in both kidneys or more than one tumor at a time. Type 1 PRCC is the more common subtype.

Type 2 PRCC tends to grow faster and is more likely to spread beyond the kidney to other parts of the body — a process known as metastasis. When cancer spreads, it can be harder to find effective treatments. A distinct form of type 2 PRCC is now known as hereditary leiomyomatosis and renal cell cancer (HLRCC). This rare genetic condition can also cause skin and uterine tumors. Some tumors that were once labeled “type 2” are now recognized as separate types of cancer with their own genetic and clinical patterns.

3. Papillary Renal Cell Carcinoma Has Several Risk Factors

PRCC can affect anyone, but certain factors may raise the risk. Some cases are sporadic, which means they happen by chance with no clear cause. Others are hereditary, or passed down through families through genetic mutations (changes in DNA).

PRCC is more common in men, according to the journal Cancers, and most people are diagnosed in their 50s, 60s, or 70s. Researchers are still studying the exact causes of sporadic PRCC, but they’ve found possible links to:

• High body weight
• Cigarette smoking or tobacco use
• Hypertension (high blood pressure)
• Chronic kidney disease
• Long-term dialysis (a treatment that filters blood when the kidneys don’t work properly)

Additionally, if you have a known family history of kidney cancer or inherited syndromes, your doctor may suggest genetic testing to better understand your personal risk. One known genetic risk is HLRCC, which is caused by a mutation in the FH gene. HLRCC can lead to a more aggressive form of type 2 PRCC that may be diagnosed at a younger age. Another condition, hereditary papillary renal cancer — which is linked to type 1 PRCC — results from changes in the MET gene.

4. Symptoms May Not Be Obvious at First

Many times, PRCC doesn’t cause obvious symptoms in the early stages. Many people are diagnosed by accident during imaging tests, such as CT scans or ultrasound, for other health concerns. Sometimes, symptoms are mistaken for more common issues, such as kidney stones or urinary tract infections. This happens because these early kidney tumors often grow slowly and silently, without causing pain or noticeable changes.

As the tumor gets larger or begins to spread, symptoms may start to appear. Common symptoms of PRCC can include:

• Hematuria (blood in the urine, which may look pink, red, or tea- or rust-colored)
• Pain, cramps, or pressure in the lower back or side
• A mass (lump) in your side, belly, or lower back
• Unexplained weight loss
• Low energy or fatigue (extreme tiredness that persists despite rest)
• Ongoing fever or a persistent cough with no clear cause

Up to 25 percent to 30 percent of people with PRCC don’t realize they have it until it has spread to other parts of the body. If something feels off or unusual in your body, tell your healthcare team. They can do imaging tests, blood tests, or a biopsy (removing and examining a small tissue sample) to find out what’s going on.

5. Treatment Depends on Disease Stage and Spread

PRCC treatment depends on how large the tumor is and whether it has spread, as well as on your overall health.

If the cancer is localized (only in the kidney), surgery is usually the first step. If the tumor is small and in an easier-to-reach area, your doctor may recommend a partial nephrectomy — removing just the tumor and some surrounding tissue. If the tumor is larger or in a tricky spot, a radical nephrectomy may be needed. This procedure removes the entire kidney along with nearby tissue, such as fat, lymph nodes, or the adrenal gland (which sits on top of the kidney).

If surgery isn’t safe for you, your doctor may recommend active surveillance — a “watch-and-wait” approach with regular monitoring. Another option may be to shrink or remove the tumor with a less invasive method, such as radiofrequency ablation, which uses targeted heat to shrink and kill tumor cells.

If the cancer has spread to other parts of the body (called metastatic PRCC), treatment focuses on slowing the disease and easing symptoms. Options may include:

• Immunotherapy — Medications that help your immune system attack the cancer cells
• Targeted therapy — Drugs that block signals cancer cells use to grow and spread
• Clinical trials — Research studies that offer access to potential treatments that aren’t widely available

Your oncology (cancer) team will guide you through your treatment options based on your cancer stage (how far the cancer has spread), your test results, and your personal needs. If you’re interested in taking part in clinical trials, they can help you find studies you may be eligible to join.

6. Outlook Depends on the PRCC Type and Stage

The prognosis for people with PRCC varies based on several factors, especially the type of PRCC and how far the cancer has spread at the time of diagnosis.

Because type 1 PRCC tends to grow more slowly and respond better to treatment, people with this type tend to have a more favorable outcome. Type 2 is usually more aggressive and harder to treat, especially if it’s diagnosed at a later stage. The stage of the cancer — whether it’s confined to the kidney or has spread — plays a large role in predicting outcomes for both types. People with early-stage PRCC often do well after surgery.

Living with PRCC is about more than just treating the cancer — it’s also about caring for your whole self. If you need support, reach out to your healthcare team, a counselor, or a trusted friend or family member. Staying connected, asking questions, and focusing on your mental and physical health can help you feel more in control and make a real difference in your quality of life.

Talk With Others Who Understand

On MyKidneyCancerTeam, people with kidney disease and their loved ones come together to ask questions, give advice, and share their stories with others who understand life with kidney disease.

Have you been diagnosed with papillary renal cell carcinoma? What tests led to your diagnosis? Share your story in the comments below, or start a conversation by posting on your Activities page.