Transitional cell cancer of the renal pelvis and ureter is a rare urinary tract cancer that typically affects adults ages 65 and older.

The urinary tract is made up of the kidneys, the ureters, the bladder, and the urethra. Urine is made in the kidney cortex and then collects in the center of the kidney, called the renal pelvis, before draining into the ureters, the tubes that connect the kidney to the bladder. Once enough urine collects in the bladder, a person will urinate—the urine goes out of the body through the urethra.

There is a dense mat of cells that lines the entire urinary tract, from the renal pelvis to the ureters, the bladder, and then into most of the urethra. Those cells are called transitional cells or urothelium. Greater than 90% of cancers in these areas come from transitional cells that have become cancerous and grow in an uncontrolled way. When that happens, instead of a flat lining, masses form in the urinary tract, which can disrupt urine flow, cause pain, and lead to blockages and/or bleeding in the urinary tract.

The good news is that when transitional cell cancer of the renal pelvis and ureter is caught early, it’s very treatable. When the cancer spreads, however, long-term survival rates taper off, depending on how far the cancer has traveled within the body.

“Because these upper tract cancers come from the same types of cells that line the bladder, we treat them the way we treat bladder cancers, not the way we treat other kidney cancers,” says Yale Medicine oncologist Michael Hurwitz, MD, PhD. “We now have a large number of treatments, ranging from surgery to immune therapies to chemotherapies, including combinations.”

These transitional cell cancers (also called urothelial cancers or urothelial carcinomas) can develop anywhere in the urinary tract including in the renal pelvis or the ureters. When they occur in the renal pelvis or ureters they are called upper tract cancers. Transitional cell cancer of the upper tract is uncommon. They account for about 10% of all transitional cell cancers.

Doctors don’t yet know the exact cause of transitional cell cancer of the renal pelvis and ureter. Though genetics seem to be a factor, it also appears that environmental factors, including smoking and exposure to certain chemicals, raise risk as well.

Symptoms of this type of cancer include:

• Blood in the urine
• Back pain
• Cramps in the side or back
• Fatigue
• Unexplained weight loss
• Pain or burning with urination
• Frequent urination

People who are at increased risk of this kind of cancer may have a combination of one or more of the following:

• A history of bladder cancer
• Lynch syndrome

Kidney damage caused by the painkiller phenacetin is a risk factor for transitional cell cancer, but phenacetin been off the market since the late 1980s and was not frequently used in the U.S. No other painkillers have been associated with transitional cell cancer.

Additionally, risk increases among people who:

• Are smokers
• Have been exposed to chemicals and/or dyes that are used in the manufacturing process to create plastics, rubber, printed materials, leather goods, and textiles

To diagnose transitional cell cancer of the renal pelvis and ureter, doctors will obtain a medical history, perform a physical exam, and order diagnostic tests.

During a medical history, doctors might ask about your personal history of cancer or of Lynch syndrome. They will also review your list of medications, as well as ask about smoking status and whether you have been exposed to potentially hazardous chemicals.

Sometimes, a doctor may be able to feel a mass during the physical exam, but typically diagnosis will occur after one or more of the following tests are performed.  

• Imaging tests. A CT scan, MRI, or ultrasound may reveal a tumor or mass.
• Urinalysis, which checks for the presence of blood and abnormal levels of protein, sugar, and bacteria, in the urine.
• Urine cytology, in which a urine sample is examined under a microscope to look for the presence of cancer cells.
• Ureteroscopy. Doctors may insert a ureteroscope (a long, narrow, flexible tube with a lens at the end) into the urethra and thread it upwards through the urinary tract to look for abnormalities
• Biopsy. When doctors pinpoint an area within the renal pelvis or ureter that appears to be cancerous, they will remove cells from that area during a biopsy and examine them to confirm a cancer diagnosis.

• Surgery. Surgery is usually recommended as a first-line treatment to remove the affected kidney, renal pelvis, ureter, and bladder cuff (where the ureter connects to the bladder). In certain circumstances, when an individual has poorly functioning kidneys or only one kidney, kidney-sparing surgery and/or chemotherapy may be used as a treatment option.
• Chemotherapy. After surgery, patients may undergo chemotherapy to help prevent cancer from developing in the bladder. (About half of people with transitional cell cancer of the renal pelvis and ureter will later develop bladder cancer.) People with advanced disease that has spread to other parts of the body may receive chemotherapy in combination with immunotherapy.

• Radiation therapy. For patients who cannot tolerate surgery, radiation plus chemotherapy is a standard-of-care treatment.
• Targeted therapy. Doctors may use a laser to target and kill cancer cells within the renal pelvis or ureter. If a section of the ureter needs to be surgically removed, the remaining portion may be reattached to the kidney, so that it can continue to function.

Some people who have low-grade, localized transitional cell cancer of the renal pelvis and ureter may not need to have their kidneys removed. Instead, doctors may surgically remove cancer from within the renal pelvis or ureter while leaving everything in place.

The outlook for people with transitional cell cancer of the renal pelvis and ureter depends on several factors, including patient age and overall health, as well as the stage and grade of the tumor. In general, lower-stage and lower-grade cancers have a better prognosis than high-stage and high-grade cancers. It’s helpful to get an early diagnosis of transitional cell cancer of the renal pelvis and ureter because the disease is highly curable when it’s treated before it spreads. After treatment, patients should continue to see their doctor regularly to monitor the possible recurrence of the cancer.

“Upper tract transitional cell cancers are rare, so it is important to get treatment for them by doctors who are experienced in all types of transitional cell cancers,” says Dr. Hurwitz. “At Yale, we see a very large number of bladder cancers, including those in the upper tract. Also, it is very important to have a multidisciplinary team of surgeons, medical oncologists, and radiation oncologists who work together closely, which exists within the Yale urologic oncology team, to manage these complex tumors.”