Urethral cancer

Urethral cancer is a rare cancer that starts in the tube that carries urine from the bladder out of the body, called the urethra. You may hear this cancer called urethral carcinoma. Urethral cancer makes up less than 1% of cancers of the urinary and reproductive systems. People of any sex can develop it, but it is more common in males.

Cancer that starts in the urethra is called primary urethral cancer. Sometimes, cancer that started elsewhere in the urinary tract, such as the bladder, comes back in the urethra after treatment. This is a recurrence of the earlier cancer, not a new cancer that started in the urethra. Healthcare professionals may call this secondary urethral carcinoma. This guide focuses on primary urethral cancer.

Learning you have a rare cancer can bring questions and uncertainty. The outlook for urethral cancer varies from person to person. It depends on factors such as where the cancer is and how far it has grown or spread. Several treatment options are available. Your care team can help you understand which options may be right for you.

Types

The main types of urethral cancer include urothelial carcinoma, squamous cell carcinoma and adenocarcinoma.

• Urothelial carcinoma. This cancer starts in cells called urothelial cells, which line parts of the urinary tract. Urothelial carcinoma also is called transitional cell carcinoma.
• Squamous cell carcinoma. This cancer starts in squamous cells, which line parts of the urethra.
• Adenocarcinoma. This cancer starts in gland-forming cells. Some urethral adenocarcinomas develop in a urethral diverticulum, which is a pouch that forms along the urethra.

Urethral cancer may cause symptoms such as:

• Blood in the urine or bloody discharge from the urethra.
• Trouble urinating.
• A lump or mass near the urethra.
• Pain in the pelvis.
• Pain during sex.

The causes of urethral cancer aren't well understood. Certain conditions and infections have been linked to a higher risk of urethral cancer.

Several factors may increase the risk of urethral cancer. These include:

• Older age. Urethral cancer becomes more common with age.
• Infections. These include certain types of human papillomavirus (HPV).
• Long-term irritation or inflammation of the urethra. In men, conditions linked to this include a narrowing of the urethra, called urethral stricture, long-term catheter use and previous surgery to repair a urethral stricture. In women, urethral diverticulum and repeated urinary tract infections may increase risk. A urethral diverticulum is a pouch that forms along the urethra. Cancer in a urethral diverticulum is rare.

There is no known way to prevent urethral cancer. There also is no routine screening for urethral cancer in people who do not have symptoms. Tests are used when urethral cancer is suspected. Or they are used to learn more about the cancer after it has been diagnosed.

Diagnosing urethral cancer usually involves looking at the urethra and taking a tissue sample to test for cancer. Imaging tests help determine how far the cancer has grown or spread.

• Physical exam. Your healthcare professional checks the urethra and nearby areas for a lump. The groin also is checked for swollen lymph nodes.
• Urethrocystoscopy. During this test, a thin viewing instrument is passed through the urethra to look at the urethra and bladder. A tissue sample also may be taken during the procedure.
• Biopsy. A small tissue sample is removed and examined under a microscope. The sample may be taken through the urethra. A biopsy confirms whether cancer is present and helps identify the type of cancer.
• CT scan. A CT scan of the chest, abdomen and pelvis looks for signs that cancer has spread to distant parts of the body.
• MRI. An MRI gives detailed pictures of the urethral tumor and nearby tissues. It helps show the tumor's size, how far it has grown, and whether nearby structures or lymph nodes may be involved.
• Urine cytology. A sample of urine is checked for cancer cells.

Grades

Grade describes how cancer cells look under a microscope. It can give your care team information about how the cancer may behave. Different types of urethral cancer use different grading systems.

Urothelial cancers may be described as low grade or high grade. Other types of urethral cancer may be graded:

• Grade 1. The cancer cells look more like the cells they started from. A lab report may call this well differentiated.
• Grade 2. The cancer cells look somewhat different from the cells they started from. A lab report may call this moderately differentiated.
• Grade 3. The cancer cells look the least like the cells they started from. A lab report may call this poorly differentiated.

In general, a higher grade is linked with a worse outlook.

Treatment depends on where the cancer is, how far it has grown or spread, and the type of cancer. Your overall health and preferences also can affect the treatment plan. Treatment may include surgery, radiation therapy, chemotherapy or other treatments. Sometimes, more than one treatment is needed.

Because urethral cancer is rare, care at a medical center with experience treating this cancer may be helpful. Your care team may include specialists such as urologists, medical oncologists and radiation oncologists.

Urethral cancer can come back after treatment. The chance of recurrence varies depending on the cancer and the treatment used. Your care team will recommend follow-up based on your individual situation.

Surgery

Surgery is an important treatment for urethral cancer that has not spread to distant parts of the body. When possible, the goal is to remove the cancer while preserving as much healthy tissue as possible. How much tissue needs to be removed depends on where the cancer is and how far it has grown.

Surgery may differ based on anatomy and location of the tumor in the urethra. In men, some tumors near the end of the urethra can be treated by removing only the affected part of the urethra. More extensive tumors may require removal of part of the penis or removal of the urethra along with nearby organs. In women, some tumors near the urethral opening may be treated by removing part of the urethra. More extensive tumors may require removal of the urethra and bladder.

Possible complications depend on the surgery. They can include issues controlling urine and, after more extensive surgery, the need for a new way for urine to leave the body.

Radiation therapy

Radiation therapy treats cancer with powerful energy beams. It may be used on its own or combined with chemotherapy or surgery. The type and amount of radiation depend on the cancer and treatment plan.

Radiation therapy can cause side effects and complications. These may include urinary symptoms. Longer-term complications can include an abnormal opening between organs or tissues, called a fistula, or narrowing of the urethra, called a urethral stricture.

Chemotherapy

Chemotherapy treats cancer with strong medicines. It may be used when cancer has grown into nearby tissues or spread to other parts of the body. Chemotherapy also may be given before surgery or combined with radiation therapy.

The medicines used depend in part on the type of urethral cancer. For urothelial cancer, chemotherapy may be given before surgery when the cancer has grown more deeply into nearby tissues. Because urethral cancer is rare, healthcare professionals sometimes use chemotherapy based on treatments for other cancers with the same cell type.

Chemotherapy side effects depend on the medicines used. They may include nausea and vomiting, hair loss, fatigue, a higher risk of infection, kidney problems, and nerve damage that can cause numbness or tingling.

Immunotherapy

Immunotherapy uses medicines that help the immune system fight cancer. Depending on the medicine, treatment may be given through a vein or as a shot under the skin.

Immunotherapy may be an option when urethral cancer has spread to distant parts of the body, particularly when the cancer is urothelial cancer.

People with urethral cancer have been included in studies of immunotherapy for urothelial cancer. But the studies have not reported results specifically for people with urethral cancer, so it isn't clear how well immunotherapy works for this cancer.

Immunotherapy can cause side effects when the immune system also affects healthy tissues or organs. These effects can include fatigue and skin rash.

Treatment for cancer that has grown or spread

Cancer that has grown into nearby tissues often needs more than one treatment. Depending on the type of cancer, treatment may include chemotherapy before surgery or chemotherapy and radiation therapy given together. Surgery may follow if it is needed.

If cancer has spread to distant parts of the body, medicines are usually the main treatment. Treatment may include medicines such as enfortumab vedotin (Padcev) with pembrolizumab (Keytruda), depending on the type of urethral cancer. Chemotherapy also may be an option. The treatment recommended also depends on factors such as your overall health and treatments you've already received.

Palliative care

Palliative care can help manage symptoms and other concerns related to cancer and its treatment. It also can help with stress, coping and making treatment decisions. Palliative care can be given at the same time as cancer treatment.

Experts recommend involving a palliative care team early when cancer has spread or when symptoms or quality-of-life concerns are difficult to manage.

Learning you have urethral cancer can bring a lot of new information and difficult decisions. As you adjust to the diagnosis and treatment, these ideas may help:

Learn about your cancer and treatment

Ask your care team to explain the type, grade and stage of your cancer and how these affect your treatment choices. If something isn't clear, ask for medical terms to be explained in plain language.

Because urethral cancer is rare, you also may want to ask whether getting care at a center experienced in treating urethral cancer would be helpful.

Ask people you trust for support

Decide whether you want a family member or friend involved in appointments or treatment decisions. Tell your care team what role you'd like that person to have.

Ask for more support if you need it

If cancer is causing emotional, social or other concerns, tell your care team. Support may be available from professionals such as social workers, psychologists, spiritual care providers or a palliative care team.

Preparing a list of questions can help you make the most of your appointment.

What you can do

• Write down the questions that are most important to you.
• Decide whether you want a family member or friend to join the discussion.
• Ask your healthcare professional to explain unfamiliar medical terms in plain language.

Questions you may want to ask include:

• What type and grade of urethral cancer do I have?
• What is the stage of my cancer? Has it reached nearby lymph nodes or other parts of my body?
• Do I need any more tests before treatment starts?
• What are the goals of my treatment?
• Which treatments do you recommend for me, and why?
• Will I need more than one type of treatment?
• How could treatment affect urination or sexual function?
• What side effects or complications should I expect?
• Should I consider a second opinion or care at a center experienced in treating urethral cancer?
• What do the survival statistics mean for someone with my type and stage of cancer?
• How soon do I need to decide about treatment?

Staging describes how far cancer has grown or spread. The stage helps your care team plan treatment. Urethral cancer uses a staging system called TNM.

The TNM system looks at the tumor, nearby lymph nodes and whether cancer has spread to distant parts of the body.

• Ta or Tis. The tumor is limited to the inner lining of the urethra.
• T1. The tumor has grown into tissue beneath the lining of the urethra.
• T2. The tumor has grown into tissues next to the urethra, such as the prostate.
• T3. The tumor has grown farther into nearby structures, such as the front wall of the vagina or the bladder neck.
• T4. The tumor has grown into another nearby organ.
• N0. There is no evidence of cancer in lymph nodes.
• N1. Cancer is found in one nearby lymph node.
• N2. Cancer is found in more than one nearby lymph node.
• M0. There is no evidence of cancer in distant parts of the body.
• M1. Cancer has spread to a distant part of the body.

Results from imaging tests, including CT and MRI, help your care team determine the cancer stage.

In one study of males with urethral cancer, the five-year overall survival was 46% and the 10-year overall survival rate was 29%. In a separate study of females with urethral cancer, the five-year overall survival rate was 43% and the 10-year overall survival rate was 32%.

Overall survival rates include deaths from urethral cancer as well as other causes. The studies included people diagnosed many years ago, so the numbers may not reflect current treatments and outcomes.

The statistics also cannot predict exactly what will happen to one person. Your outlook depends on many things, including the cancer's stage and grade, where the tumor is, and whether the cancer has spread to lymph nodes or other parts of the body. Your healthcare professional can help you understand which survival information is most relevant to your situation.