Upper urinary tract urothelial cancer

Upper urinary tract urothelial cancer is a rare type of cancer of the lining of the kidney or ureter. It used to be called transitional cell cancer (TCC) of the kidney or ureter. This is because it starts in cells called transitional cells.

There are many different types of cells in the body, each with a particular job to do. Transitional cells line the renal pelvis, ureters, bladder and urethra. They can change shape and stretch. This means these parts of the body can expand to store urine or to let urine flow through them.

The kidneys and ureters

The kidneys are two bean shaped organs, each about the size of a fist. They are near the middle of your back, one on either side of your spine. They are part of the body’s urinary system. They filter waste products out of the blood and make urine. The ureters are the tubes that carry urine from the kidney to the bladder.

Upper urinary tract urothelial cancer can start in a part of the kidney called the renal pelvis. Or it can start in the ureters.

The renal pelvis is in the middle of the kidney. Urine collects here then drains through the ureter into the bladder. When you empty your bladder, the urine leaves your body through a tube called the urethra.

How common is upper urinary tract urothelial cancer?

Upper urinary tract urothelial cancer of the kidney is rare. Upper urinary tract urothelial cancer of the ureter is even less common.

You can also get urothelial cancer of the bladder. The bladder is where urine collects before you pee it out. It is in the lower urinary tract. Urothelial cancer of the bladder is the most common type of bladder cancer.

Risks and causes of upper urinary tract urothelial cancer

Anything that can increase your risk of getting a disease is called a risk factor. Different cancers have different risk factors. Having one or more risk factors doesn't mean you will definitely get that cancer.

Upper urinary tract urothelial cancer is more common in older people. It's also diagnosed more often in men than women.

Other factors that increase your risk of upper urinary tract urothelial cancer include:

• smoking
• aristolochic acid - this is produced by aristolochia plants. It may be found in some herbal remedies and can cause kidney damage
• Lynch syndrome – this inherited syndrome increases the risk of several cancers, including urothelial cancer
• if you have bladder cancer or have had it in the past

Symptoms of upper urinary tract urothelial cancer

The symptoms of upper urinary tract urothelial cancer include:

• blood in your urine
• pain in your side between the ribs and the hip (flank)

They are similar to the symptoms of kidney cancer.

Sometimes upper urinary tract urothelial cancer is found when you’re having a scan for something else.

Getting diagnosed with upper urinary tract urothelial cancer

Your GP will examine you and test your urine for blood. You may also have blood tests to check your general health.

They may refer you to a specialist doctor (urologist) for further tests to find out the cause of your symptoms. These tests might include:

• a test using a CT scan and special dye (contrast medium) to look at the urinary system (CT urogram)
• a CT scan of your chest
• using a thin flexible tube to look inside your bladder (cystoscopy) or your ureter and kidney (ureteroscopy) to check for abnormal areas - your doctor may take a small piece of tissue (biopsy) to check for cancer
• looking for cancer cells in your urine – your doctor sends a sample of your urine to the laboratory to be looked at under a microscope

Occasionally your doctor may want you to have other tests. They will explain what the tests are for and how to prepare for them.

Treatment for upper urinary tract urothelial cancer

Your doctor considers many factors when deciding which treatment is suitable for you. This includes:

• the size of the cancer and whether it has spread to other parts of your body
• how abnormal the cancer cells look under the microscope (the grade)
• how well your kidneys work
• your general health

Surgery is the most common treatment for upper urinary tract urothelial cancer. This is a big operation, so your doctor will check if you’re healthy enough to make a good recovery.

If you have a small cancer that has a low risk of spreading, or your kidneys don’t work very well, you may be able to have a smaller procedure. This means you don’t need to have your kidney removed. This is only possible for a very small number of people. Your doctor will tell you if this is suitable for you.

If the cancer is advanced, or you can’t have surgery, you may have chemotherapy or immunotherapy instead of an operation.

Your doctor will talk to you about the treatment options, including the benefits and possible side effects. Some people find it helpful to have a relative or friend with them when they see their doctor. They can:

• support you
• ask questions
• take notes for you

You may find it helpful to talk to your specialist nurse (CNS) about any worries you have about the treatments before you make your decision.

Surgery

You usually have surgery if the cancer hasn't spread, and you’re well enough. Your surgeon removes your kidney, ureter and part of your bladder. This is called a radical nephroureterectomy (neff-roh-your-ee-ter-eck-toh-mee).

They might also remove some of the lymph nodes near your kidney and some surrounding tissue.

If the cancer is in your ureter, your surgeon may only remove the part with the cancer and not the kidney. This operation has a higher risk of the cancer coming back. So, it’s only recommended for a small number of people with cancer that has a low chance of spreading.

Laser or heat treatment (endoscopic ablation)

For some very small cancers that have a lower chance of spreading, you may have heat or laser treatment to treat the cancer. Your doctor puts a small flexible tube up into your bladder to the ureter. They then use heat or a laser to kill the cancer cells.

Your doctor and CNS will talk with you about preparing for this procedure and how long it may take to recover. They will also talk with you about any side effects you may get and how to manage them.

There is a risk the cancer may come back after this treatment. So, you have regular follow up appointments to check the area.

Chemotherapy after surgery

Your doctor may recommend you have chemotherapy after surgery if the cancer has:

• grown into the layer of muscle or deeper
• spread to the nearby lymph nodes

This is to reduce the risk of the cancer coming back. It’s called adjuvant chemotherapy.

Chemotherapy uses anti cancer (cytotoxic) drugs to destroy cancer cells. You usually have 2 chemotherapy drugs. These may be either:

• gemcitabine with cisplatin (GC)
• gemcitabine with carboplatin (GemCarbo)

Having chemotherapy means that you have to go to the hospital a number of times each month. Tell your doctor and CNS if this might be difficult. They will talk with you about any support that is available.

Having GC or GemCarbo

You have chemotherapy as cycles of treatment. Each cycle of GC or GemCarbo takes 3 weeks, and you have 4 cycles. On:

• day 1 – you have gemcitabine and cisplatin or carboplatin
• day 8 – you have gemcitabine again

At the end of 3 weeks (21 days) you start a new cycle and go back to day 1.

You have the treatment as a drip into your bloodstream (intravenously). There are different ways to have these drugs.

If you have a medical condition that means you can’t have chemotherapy, you may have an immunotherapy drug called nivolumab after surgery. But only if the cancer cells have a protein called PD-L1 on their surface.

PD-L1 stops the immune system from working properly and attacking cancer cells. Immunotherapy drugs like nivolumab help your immune system find and kill the cancer cells.

Chemotherapy into the bladder (intravesical chemotherapy)

If you have a nephroureterectomy your doctor may give you a single dose of chemotherapy straight into your bladder. This is called intravesical chemotherapy. You normally have this a few days after your operation while you are still in hospital. It reduces the risk of the cancer coming back in your bladder.

The chemotherapy comes out in your urine. It drains into a bag if you have a catheter in your bladder. You need to be careful to stop the pee touching your skin. Your nurse will explain how to do this.

Treatment for advanced upper urinary tract urothelial cancer

Advanced upper urinary tract urothelial cancer means that the cancer has grown outside the kidney or ureter (locally advanced). Or has spread to another part of the body (metastatic cancer). The main treatments are chemotherapy and immunotherapy.

Chemotherapy

You may have either:

• gemcitabine with cisplatin (GC)
• gemcitabine with carboplatin (GemCarbo)

Occasionally your doctor might recommend you have a combination of the chemotherapy drugs methotrexate, vinblastine, doxorubicin and cisplatin (MVAC).

Immunotherapy

If the chemotherapy works well, after 4 to 6 cycles you might have an immunotherapy drug called avelumab. This is to try and control the cancer for longer. It’s called a maintenance treatment.  

You have avelumab through a drip into your bloodstream every 2 weeks. You can have it for up to 5 years.

You might have a different immunotherapy drug called atezolizumab (Tecentriq) if:

• you can’t have cisplatin or carboplatin chemotherapy, and
• the cancer cells have PD-L1 protein on their surface

You might also have atezolizumab if the cancer cells don’t have PD-L1 on their surface and:

• you haven’t had an immunotherapy drug already, and
• the cancer has started growing again after chemotherapy

Radiotherapy

Radiotherapy isn't often given for upper urinary tract urothelial cancer. You may have it after surgery if the cancer has spread to the tissue around the kidney.

A very small number of people may have radiotherapy if the cancer has spread to the tissue around the ureter.

Follow up for upper urinary tract urothelial cancer

You have regular follow up appointments after your treatment.

If the cancer hasn’t spread

You usually have appointments:

• every 3-6 months during the first year
• every 6 months in the second and third year
• yearly afterwards

At these appointments your doctor looks for signs of the cancer coming back or spreading so it can be treated early.

As part of your follow ups you have a test to look inside your bladder (cystoscopy). This is because upper urinary tract urothelial cancer can sometimes come back in your bladder. You may also have CT scans.

Advanced cancer

If you have advanced upper urinary tract urothelial cancer, when and how often you see your doctor or nurse specialist will vary. This depends on several things including:

• how well the treatment is working
• if there are other treatments you can have
• if you’re taking part in a clinical trial
• what symptoms you have

You may also be under the care of doctors and nurses who are experts in symptom control.

It is important that you do not miss your tests and follow up appointments. Make sure you know who to contact if you need to change the date or need help getting to the hospital.

If you move out of the area:

• tell your old hospital as soon as you know where you are moving to – depending on where you are going, they might be able to transfer your care to a nearby hospital
• tell your new GP that you are being followed up for upper urinary tract urothelial cancer
• keep hold of the contact details for your CNS at the old hospital – you may need to talk to them about getting your information to your new doctor
• make sure your contact details are up to date

Research into upper urinary tract urothelial cancer

Researchers are looking into developing new treatments and improving existing treatments for upper urinary tract urothelial cancer. These are called clinical trials. If you are interested in joining a clinical trial discuss it with your doctor.

Coping with upper urinary tract urothelial cancer

Coping with a diagnosis of a rare cancer can be especially difficult. Being well informed about your cancer and its treatment can help. It can make it easier to make decisions and cope with what happens.

Your CNS and their team are a good source of support. They can talk with you about any support services for you and your family. And they can refer you specialist teams to help you cope.

Talking to other people who have the same thing can also help.

Our discussion forum Cancer Chat is a place for anyone affected by cancer. You can share experiences, stories and information with other people.

The Rare Cancer Alliance offer support and information to people with rare cancers.

You can find more resources in the kidney and bladder cancer sections of the Cancer Research UK website.