Vaginal Cancer—Patient Version

Vaginal Cancer—Patient Version

Overview

Infection with human papillomavirus (HPV) causes two-thirds of the cases of vaginal cancer. Vaccines that protect against infection with HPV may reduce the risk of vaginal cancer. When found early, vaginal cancer can often be cured. Explore the links on this page to learn more about vaginal cancer treatment, research, and clinical trials.

Causes & Prevention

NCI does not have PDQ evidence-based information about prevention of vaginal cancer.

Screening

NCI does not have PDQ evidence-based information about screening for vaginal cancer.

Coping with Cancer

The information in this section is meant to help you cope with the many issues and concerns that occur when you have cancer.

Emotions and Cancer Adjusting to Cancer Support for Caregivers Survivorship Advanced Cancer Managing Cancer Care

Urethral Cancer Treatment (PDQ®)–Patient Version

Urethral Cancer Treatment (PDQ®)–Patient Version

General Information About Urethral Cancer

Key Points

  • Urethral cancer is a disease in which malignant (cancer) cells form in the tissues of the urethra.
  • There are different types of urethral cancer that begin in cells that line the urethra.
  • A history of bladder cancer can affect the risk of urethral cancer.
  • Signs of urethral cancer include bleeding or trouble with urination.
  • Tests that examine the urethra and bladder are used to diagnose urethral cancer.
  • Certain factors affect prognosis (chance of recovery) and treatment options.

Urethral cancer is a disease in which malignant (cancer) cells form in the tissues of the urethra.

The urethra is the tube that carries urine from the bladder to outside the body. In women, the urethra is about 1½ inches long and is just above the vagina. In men, the urethra is about 8 inches long, and goes through the prostate gland and the penis to the outside of the body. In men, the urethra also carries semen.

EnlargeAnatomy of the male urinary system (left panel) and female urinary system (right panel); two-panel drawing showing the right and left kidneys, the ureters, the bladder filled with urine, and the urethra. The inside of the left kidney shows the renal pelvis. An inset shows the renal tubules and urine. Also shown are the prostate and penis (left panel) and the uterus (right panel).
Anatomy of the male urinary system (left panel) and female urinary system (right panel) showing the kidneys, ureters, bladder, and urethra. The inside of the left kidney shows the renal pelvis. An inset shows the renal tubules and urine. Also shown are the prostate and penis (left panel) and the uterus (right panel). Urine is made in the renal tubules and collects in the renal pelvis of each kidney. The urine flows from the kidneys through the ureters to the bladder. The urine is stored in the bladder until it leaves the body through the urethra.

Urethral cancer is a rare cancer that occurs more often in men than in women.

There are different types of urethral cancer that begin in cells that line the urethra.

These cancers are named for the types of cells that become malignant (cancer):

  • Squamous cell carcinoma is the most common type of urethral cancer. It forms in the thin, flat cells in the part of the urethra near the bladder in women, and in the lining of the urethra in the penis in men.
  • Transitional cell carcinoma forms in the area near the urethral opening in women, and in the part of the urethra that goes through the prostate gland in men.
  • Adenocarcinoma forms in the glands that are around the urethra in both men and women.

Urethral cancer can metastasize (spread) quickly to tissues around the urethra and is often found in nearby lymph nodes by the time it is diagnosed.

A history of bladder cancer can affect the risk of urethral cancer.

Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for urethral cancer include the following:

Signs of urethral cancer include bleeding or trouble with urination.

These and other signs and symptoms may be caused by urethral cancer or by other conditions. There may be no signs or symptoms in the early stages. Check with your doctor if you have any of the following:

  • Trouble starting the flow of urine.
  • Weak or interrupted (“stop-and-go”) flow of urine.
  • Frequent urination, especially at night.
  • Incontinence.
  • Discharge from the urethra.
  • Bleeding from the urethra or blood in the urine.
  • A lump or thickness in the perineum or penis.
  • A painless lump or swelling in the groin.

Tests that examine the urethra and bladder are used to diagnose urethral cancer.

The following tests and procedures may be used:

  • Physical exam and health history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
  • Pelvic exam: An exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. A speculum is inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. The doctor or nurse also inserts one or two lubricated, gloved fingers of one hand into the vagina and places the other hand over the lower abdomen to feel the size, shape, and position of the uterus and ovaries. The doctor or nurse also inserts a lubricated, gloved finger into the rectum to feel for lumps or abnormal areas.
    EnlargePelvic exam; drawing shows a side view of the female reproductive anatomy during a pelvic exam. The uterus, left fallopian tube, left ovary, cervix, vagina, bladder, and rectum are shown. Two gloved fingers of one hand of the doctor or nurse are shown inserted into the vagina, while the other hand is shown pressing on the lower abdomen. The inset shows a woman covered by a drape on an exam table with her legs apart and her feet in stirrups.
    Pelvic exam. A doctor or nurse inserts one or two lubricated, gloved fingers of one hand into the vagina and presses on the lower abdomen with the other hand. This is done to feel the size, shape, and position of the uterus and ovaries. The vagina, cervix, fallopian tubes, and rectum are also checked.
  • Digital rectal exam: An exam of the rectum. The doctor or nurse inserts a lubricated, gloved finger into the lower part of the rectum to feel for lumps or anything else that seems unusual.
  • Urine cytology: A laboratory test in which a sample of urine is checked under a microscope for abnormal cells.
  • Urinalysis: A test to check the color of urine and its contents, such as sugar, protein, blood, and white blood cells. If white blood cells (a sign of infection) are found, a urine culture is usually done to find out what type of infection it is.
  • Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
  • Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the pelvis and abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
  • Ureteroscopy: A procedure to look inside the ureter and renal pelvis to check for abnormal areas. A ureteroscope is a thin, tube-like instrument with a light and a lens for viewing. The ureteroscope is inserted through the urethra into the bladder, ureter, and renal pelvis. A tool may be inserted through the ureteroscope to take tissue samples to be checked under a microscope for signs of disease.
    EnlargeDrawing of a female and male ureteroscopy showing the lower pelvis of a female, including the right and left kidneys, renal pelvis, ureter, uterus, bladder, and urethra, and the lower pelvis of a male, including the ureter, bladder, prostate, urethra, and penis. In both the female and male figures, a ureteroscope (a thin, tube-like instrument with a light and a lens for viewing) is shown passing through the urethra into the bladder and ureter. In the female figure, the ureteroscope is also shown passing into the renal pelvis. There is also an inset that shows a person lying on an examination table with their knees bent and legs apart, covered by a drape, and a doctor looking at an image of the inside of the ureter and/or renal pelvis on a computer monitor.
    A ureteroscopy is a procedure that uses a ureteroscope (a thin, tube-like instrument with a light and a lens for viewing) to look inside the ureter and renal pelvis to check for abnormal areas. The ureteroscope is inserted through the urethra into the bladder, ureter, and renal pelvis.
  • Biopsy: The removal of cell or tissue samples from the urethra, bladder, and, sometimes, the prostate gland. The samples are viewed under a microscope by a pathologist to check for signs of cancer.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis and treatment options depend on the following:

  • Where the cancer formed in the urethra.
  • Whether the cancer has spread through the mucosa lining the urethra to nearby tissue, to lymph nodes, or to other parts of the body.
  • Whether the patient is a male or female.
  • The patient’s general health.
  • Whether the cancer has just been diagnosed or has recurred (come back).

Stages of Urethral Cancer

Key Points

  • After urethral cancer has been diagnosed, tests are done to find out if cancer cells have spread within the urethra or to other parts of the body.
  • There are three ways that cancer spreads in the body.
  • Cancer may spread from where it began to other parts of the body.
  • Urethral cancer is staged and treated based on the part of the urethra that is affected.
    • Distal urethral cancer
    • Proximal urethral cancer
  • Bladder and/or prostate cancer may occur at the same time as urethral cancer.
  • Urethral cancer can recur (come back) after it has been treated.

After urethral cancer has been diagnosed, tests are done to find out if cancer cells have spread within the urethra or to other parts of the body.

The process used to find out if cancer has spread within the urethra or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment.

The following procedures may be used in the staging process:

  • Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
  • CT scan (CAT scan) of the pelvis and abdomen: A procedure that makes a series of detailed pictures of the pelvis and abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of the urethra, nearby lymph nodes, and other soft tissue and bones in the pelvis. A substance called gadolinium is injected into the patient through a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI).
  • Urethrography: A series of x-rays of the urethra. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. A dye is injected through the urethra into the bladder. The dye coats the bladder and urethra and x-rays are taken to see if the urethra is blocked and if cancer has spread to nearby tissue.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

  • Tissue. The cancer spreads from where it began by growing into nearby areas.
  • Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
  • Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

  • Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
  • Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if urethral cancer spreads to the lung, the cancer cells in the lung are actually urethral cancer cells. The disease is metastatic urethral cancer, not lung cancer.

Many cancer deaths are caused when cancer moves from the original tumor and spreads to other tissues and organs. This is called metastatic cancer. This animation shows how cancer cells travel from the place in the body where they first formed to other parts of the body.

Urethral cancer is staged and treated based on the part of the urethra that is affected.

Urethral cancer is staged and treated based on the part of the urethra that is affected and how deeply the tumor has spread into tissue around the urethra. Urethral cancer can be described as distal or proximal.

EnlargeDistal and proximal urethra. Drawing on the left shows the female proximal and distal urethra; also shown are the bladder filled with urine, the ureters, and the uterus. Cross-section drawing on the right shows the male proximal and distal urethra. Also shown are the rectum, prostate gland, penis, and testis.
Anatomy of the distal and proximal urethra. Urine flows out of the bladder and leaves the body through the urethra. The part of the urethra that is closest to the bladder is called the proximal urethra. The part that is closest to where the urine leaves the body is called the distal urethra. The urethra is about 8 inches long in men and about 1½ inches long in women.

Distal urethral cancer

In distal urethral cancer, the cancer usually has not spread deeply into the tissue. In women, the part of the urethra that is closest to the outside of the body (about ½ inch) is affected. In men, the part of the urethra that is in the penis is affected.

Proximal urethral cancer

Proximal urethral cancer affects the part of the urethra that is not the distal urethra. In women and men, proximal urethral cancer usually has spread deeply into tissue.

Bladder and/or prostate cancer may occur at the same time as urethral cancer.

In men, cancer that forms in the proximal urethra (the part of the urethra that passes through the prostate to the bladder) may occur at the same time as cancer of the bladder and/or prostate. Sometimes this occurs at diagnosis and sometimes it occurs later.

Urethral cancer can recur (come back) after it has been treated.

The cancer may come back in the urethra or in other parts of the body.

Treatment Option Overview

Key Points

  • There are different types of treatment for patients with urethral cancer.
  • The following types of treatment are used:
    • Surgery
    • Radiation therapy
    • Chemotherapy
    • Active surveillance
  • New types of treatment are being tested in clinical trials.
  • Treatment for urethral cancer may cause side effects.
  • Patients may want to think about taking part in a clinical trial.
  • Patients can enter clinical trials before, during, or after starting their cancer treatment.
  • Follow-up tests may be needed.

There are different types of treatment for patients with urethral cancer.

Different types of treatments are available for patients with urethral cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

The following types of treatment are used:

Surgery

Surgery to remove the cancer is the most common treatment for cancer of the urethra. One of the following types of surgery may be done:

  • Open excision: Removal of the cancer by surgery.
  • Transurethral resection (TUR): Surgery to remove the cancer using a special tool inserted into the urethra.
  • Electroresection with fulguration: Surgery to remove the cancer by electric current. A lighted tool with a small wire loop on the end is used to remove the cancer or to burn the tumor away with high-energy electricity.
  • Laser surgery: A surgical procedure that uses a laser beam (a narrow beam of intense light) as a knife to make bloodless cuts in tissue or to remove or destroy tissue.
  • Lymph node dissection: Lymph nodes in the pelvis and groin may be removed.
  • Cystourethrectomy: Surgery to remove the bladder and the urethra.
  • Cystoprostatectomy: Surgery to remove the bladder and the prostate.
  • Anterior exenteration: Surgery to remove the urethra, the bladder, and the vagina. Plastic surgery may be done to rebuild the vagina.
  • Partial penectomy: Surgery to remove the part of the penis surrounding the urethra where cancer has spread. Plastic surgery may be done to rebuild the penis.
  • Radical penectomy: Surgery to remove the entire penis. Plastic surgery may be done to rebuild the penis.

If the urethra is removed, the surgeon will make a new way for the urine to pass from the body. This is called urinary diversion. If the bladder is removed, the surgeon will make a new way for urine to be stored and passed from the body. The surgeon may use part of the small intestine to make a tube that passes urine through an opening (stoma). This is called an ostomy or urostomy. If a patient has an ostomy, a disposable bag to collect urine is worn under clothing. The surgeon may also use part of the small intestine to make a new storage pouch (continent reservoir) inside the body where the urine can collect. A tube (catheter) is then used to drain the urine through a stoma.

After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy:

The way the radiation therapy is given depends on the type of cancer and where the cancer formed in the urethra. External and internal radiation therapy are used to treat urethral cancer.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type of cancer and where the cancer formed in the urethra.

Active surveillance

Active surveillance is following a patient’s condition without giving any treatment unless there are changes in test results. It is used to find early signs that the condition is getting worse. In active surveillance, patients are given certain exams and tests, including biopsies, on a regular schedule.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI website.

Treatment for urethral cancer may cause side effects.

For information about side effects caused by treatment for cancer, visit our Side Effects page.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today’s standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

Follow-up tests may be needed.

As you go through treatment, you will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back).

Treatment of Distal Urethral Cancer

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of abnormal cells in the mucosa (inside lining of the urethra that have not become cancer, may include surgery to remove the tumor (open excision or transurethral resection), electroresection with fulguration, or laser surgery.

Treatment of distal urethral cancer is different for men and women.

For women, treatment may include the following:

For men, treatment may include the following:

  • Surgery to remove the tumor (transurethral resection), electroresection and fulguration, or laser surgery for tumors that have not spread deeply into tissue.
  • Surgery to remove part of the penis (partial penectomy) for tumors that are near the tip of the penis. Sometimes nearby lymph nodes are also removed (lymph node dissection).
  • Surgery to remove part of the urethra for tumors that are in the distal urethra but not at the tip of the penis and have not spread deeply into tissue. Sometimes nearby lymph nodes are also removed (lymph node dissection).
  • Surgery to remove the penis (radical penectomy) for tumors that have spread deeply into tissue. Sometimes nearby lymph nodes are also removed (lymph node dissection).
  • Radiation therapy with or without chemotherapy.
  • Chemotherapy given together with radiation therapy.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Proximal Urethral Cancer

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of proximal urethral cancer or urethral cancer that affects the entire urethra is different for men and women.

For women, treatment may include the following:

For men, treatment may include the following:

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Urethral Cancer that Forms with Invasive Bladder Cancer

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of urethral cancer that forms at the same time as invasive bladder cancer may include the following:

If the urethra is not removed during surgery to remove the bladder, treatment may include the following:

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Metastatic or Recurrent Urethral Cancer

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of urethral cancer that has metastasized (spread to other parts of the body) is usually chemotherapy.

Treatment of recurrent urethral cancer may include one or more of the following:

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

To Learn More About Urethral Cancer

About This PDQ Summary

About PDQ

Physician Data Query (PDQ) is the National Cancer Institute’s (NCI’s) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.

PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of urethral cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

Reviewers and Updates

Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary (“Updated”) is the date of the most recent change.

The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.

Clinical Trial Information

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become “standard.” Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Clinical trials can be found online at NCI’s website. For more information, call the Cancer Information Service (CIS), NCI’s contact center, at 1-800-4-CANCER (1-800-422-6237).

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The best way to cite this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Urethral Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/urethral/patient/urethral-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389384]

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Urethral Cancer Treatment (PDQ®)–Health Professional Version

Urethral Cancer Treatment (PDQ®)–Health Professional Version

General Information About Urethral Cancer

Incidence and Mortality

Urethral cancer is rare. The annual incidence rate for urethral cancer in the Surveillance, Epidemiology, and End Results (SEER) Program database from 1973 to 2002 in the United States was 4.3 per million for men and 1.5 per million for women, with downward trends over the three decades.[1] The incidence was twice as high in African American individuals as in White individuals (5 per million vs. 2.5 per million). Urethral cancers appear to be associated with human papillomavirus (HPV) infection, particularly HPV16, a strain known to cause cervical cancer.[2,3]

Because urethral cancer is rare, nearly all information about its treatment and the outcomes of therapy is derived from retrospective, single-center case series, which represents a very low Level of evidence C3. Most information comes from cases accumulated over many decades at major academic centers.

Anatomy

The female urethra is largely contained within the anterior vaginal wall. In adults, it is about 4 cm in length.

The male urethra, which averages about 20 cm in length, is divided into distal and proximal portions. The distal urethra, which extends from the tip of the penis to just before the prostate, includes the meatus, the fossa navicularis, the penile or pendulous urethra, and the bulbar urethra. The proximal urethra, which extends from the bulbar urethra to the bladder neck, includes the membranous urethra and the prostatic urethra.

EnlargeAnatomy of the male urinary system (left panel) and female urinary system (right panel); two-panel drawing showing the right and left kidneys, the ureters, the bladder filled with urine, and the urethra. The inside of the left kidney shows the renal pelvis. An inset shows the renal tubules and urine. Also shown are the prostate and penis (left panel) and the uterus (right panel).
Anatomy of the male urinary system (left panel) and female urinary system (right panel) showing the kidneys, ureters, bladder, and urethra. The inside of the left kidney shows the renal pelvis. An inset shows the renal tubules and urine. Also shown are the prostate and penis (left panel) and the uterus (right panel). Urine is made in the renal tubules and collects in the renal pelvis of each kidney. The urine flows from the kidneys through the ureters to the bladder. The urine is stored in the bladder until it leaves the body through the urethra.

Prognosis

The prognosis of urethral cancer depends on the following factors:[46]

  • Anatomical location.
  • Size.
  • Stage.
  • Depth of invasion.

Superficial tumors in the distal urethra in both women and men are generally curable. However, deeply invasive lesions are rarely curable by any combination of therapies. In men, the prognosis of tumors in the distal (pendulous) urethra is better than for tumors of the proximal (bulbomembranous) and prostatic urethra, which tend to present at more advanced stages.[7,8] Likewise, distal urethral tumors tend to occur at earlier stages in women and to have a better prognosis than proximal tumors.[9]

References
  1. Swartz MA, Porter MP, Lin DW, et al.: Incidence of primary urethral carcinoma in the United States. Urology 68 (6): 1164-8, 2006. [PUBMED Abstract]
  2. Wiener JS, Liu ET, Walther PJ: Oncogenic human papillomavirus type 16 is associated with squamous cell cancer of the male urethra. Cancer Res 52 (18): 5018-23, 1992. [PUBMED Abstract]
  3. Wiener JS, Walther PJ: A high association of oncogenic human papillomaviruses with carcinomas of the female urethra: polymerase chain reaction-based analysis of multiple histological types. J Urol 151 (1): 49-53, 1994. [PUBMED Abstract]
  4. Urethra. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. Springer; 2017, pp. 767–76.
  5. Rabbani F: Prognostic factors in male urethral cancer. Cancer 117 (11): 2426-34, 2011. [PUBMED Abstract]
  6. Dalbagni G, Zhang ZF, Lacombe L, et al.: Female urethral carcinoma: an analysis of treatment outcome and a plea for a standardized management strategy. Br J Urol 82 (6): 835-41, 1998. [PUBMED Abstract]
  7. Dinney CP, Johnson DE, Swanson DA, et al.: Therapy and prognosis for male anterior urethral carcinoma: an update. Urology 43 (4): 506-14, 1994. [PUBMED Abstract]
  8. Dalbagni G, Zhang ZF, Lacombe L, et al.: Male urethral carcinoma: analysis of treatment outcome. Urology 53 (6): 1126-32, 1999. [PUBMED Abstract]
  9. Gheiler EL, Tefilli MV, Tiguert R, et al.: Management of primary urethral cancer. Urology 52 (3): 487-93, 1998. [PUBMED Abstract]

Cellular Classification of Urethral Cancer

In an analysis of Surveillance, Epidemiology, and End Results (SEER) Program data from 1973 to 2002, the most common histological types of urethral cancer were the following:[1]

  • Transitional cell (55%).
  • Squamous cell (21.5%).
  • Adenocarcinoma (16.4%).

Other cell types, such as melanoma, were extremely rare.[1]

The female urethra is lined by transitional cell mucosa proximally and stratified squamous cells distally. Therefore, transitional cell carcinoma is most common in the proximal urethra, and squamous cell carcinoma predominates in the distal urethra. Adenocarcinoma may occur in both locations and arises from metaplasia of the numerous periurethral glands.

The male urethra is lined by transitional cells in its prostatic and membranous portion and stratified columnar epithelium to stratified squamous epithelium in the bulbous and penile portions. The submucosa of the urethra contains numerous glands. Therefore, urethral cancer in men can manifest the histological characteristics of transitional cell carcinoma, squamous cell carcinoma, or adenocarcinoma.

Except for the prostatic urethra, where transitional cell carcinoma is most common, squamous cell carcinoma is the predominant histology of urethral neoplasms. Because transitional cell carcinoma of the prostatic urethra may be associated with transitional cell carcinoma of the bladder and/or transitional cell carcinoma arising in prostatic ducts, it is often treated similarly to these primaries and should be separated from the more distal carcinomas of the urethra.

References
  1. Swartz MA, Porter MP, Lin DW, et al.: Incidence of primary urethral carcinoma in the United States. Urology 68 (6): 1164-8, 2006. [PUBMED Abstract]

Stage Information for Urethral Cancer

Prognosis and treatment decisions are determined by the following:[1]

  • The anatomical location of the primary tumor.
  • The size of the tumor.
  • The stage of the cancer.
  • The depth of invasion of the tumor.

The histology of the primary tumor is of less importance in estimating response to therapy and survival.[2] Endoscopic examination, urethrography, and magnetic resonance imaging are useful in determining the local extent of the tumor.[3,4]

Distal Urethral Cancer

These lesions are often superficial.

  • Female: Lesions of the distal third of the urethra.
  • Male: Anterior, or penile, portion of the urethra, including the meatus and pendulous urethra.

Proximal Urethral Cancer

These lesions are often deeply invasive.

  • Female: Lesions not clearly limited to the distal third of the urethra.
  • Male: Bulbomembranous and prostatic urethra.

Urethral Cancer Associated With Invasive Bladder Cancer

Approximately 5% to 10% of men with cystectomy for bladder cancer may have or may develop urethral cancer distal to the urogenital diaphragm.[5,6]

American Joint Committee on Cancer (AJCC) Stage Groupings and TNM Definitions

The AJCC has designated staging by TNM (tumor, node, metastasis) classification to define urethral cancer.[1]

Male penile urethra and female urethra

Table 1. Definitions of TNM Stages 0is and 0aa
Stage TNM Description
T = primary tumor; N = regional lymph node; M = distant metastasis.
aReprinted with permission from AJCC: Urethra. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 767–76.
0is Tis, N0, M0 Tis = Carcinoma in situ.
N0 = No regional lymph node metastasis.
M0 = No distant metastasis.
0a Ta, N0, M0 Ta = Noninvasive papillary carcinoma.
N0 = No regional lymph node metastasis.
M0 = No distant metastasis.
Table 2. Definition of TNM Stage Ia
Stage TNM Description
T = primary tumor; N = regional lymph node; M = distant metastasis.
aReprinted with permission from AJCC: Urethra. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 767–76.
I T1, N0, M0 T1 = Tumor invades subepithelial connective tissue.
N0 = No regional lymph node metastasis.
M0 = No distant metastasis.
Table 3. Definition of TNM Stage IIa
Stage TNM Description
T = primary tumor; N = regional lymph node; M = distant metastasis.
aReprinted with permission from AJCC: Urethra. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 767–76.
II T2, N0, M0 T2 = Tumor invades any of the following: corpus spongiosum, periurethral muscle.
N0 = No regional lymph node metastasis.
M0 = No distant metastasis.
Table 4. Definitions of TNM Stage IIIa
Stage TNM Description
T = primary tumor; N = regional lymph node; M = distant metastasis.
aReprinted with permission from AJCC: Urethra. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 767–76.
III T1, N1, M0 T1 = Tumor invades subepithelial connective tissue.
N1 = Single regional lymph node metastasis in the inguinal region or true pelvis (perivesical, obturator, internal [hypogastric] and external iliac), or presacral lymph node.
M0 = No distant metastasis.
T2, N1, M0 T2 = Tumor invades any of the following: corpus spongiosum, periurethral muscle.
N1 = Single regional lymph node metastasis in the inguinal region or true pelvis (perivesical, obturator, internal [hypogastric] and external iliac), or presacral lymph node.
M0 = No distant metastasis.
T3, N0, M0 T3 = Tumor invades any of the following: corpus cavernosum, anterior vagina.
N0 = No regional lymph node metastasis.
M0 = No distant metastasis.
T3, N1, M0 T3 = Tumor invades any of the following: corpus cavernosum, anterior vagina.
N1 = Single regional lymph node metastasis in the inguinal region or true pelvis (perivesical, obturator, internal [hypogastric] and external iliac), or presacral lymph node.
M0 = No distant metastasis.
Table 5. Definitions of TNM Stage IVa
Stage TNM Description
T = primary tumor; N = regional lymph node; M = distant metastasis.
aReprinted with permission from AJCC: Urethra. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 767–76.
IV T4, N0, M0 T4 = Tumor invades other adjacent organs (e.g., invasion of the bladder wall).
N0 = No regional lymph node metastasis.
M0 = No distant metastasis.
T4, N1, M0 T4 = Tumor invades other adjacent organs (e.g., invasion of the bladder wall).
N1 = Single regional lymph node metastasis in the inguinal region or true pelvis (perivesical, obturator, internal [hypogastric] and external iliac), or presacral lymph node.
M0 = No distant metastasis.
Any T, N2, M0 TX = Primary tumor cannot be assessed.
T0 = No evidence of primary tumor.
Ta = Noninvasive papillary carcinoma.
Tis = Carcinoma in situ.
T1 = Tumor invades subepithelial connective tissue.
T2 = Tumor invades any of the following: corpus spongiosum, periurethral muscle.
T3 = Tumor invades any of the following: corpus cavernosum, anterior vagina.
T4 = Tumor invades other adjacent organs (e.g., invasion of the bladder wall).
N2 = Multiple regional lymph node metastasis in the inguinal region or true pelvis (perivesical, obturator, internal [hypogastric] and external iliac), or presacral lymph node.
M0 = No distant metastasis.
Any T, Any N, M1 Any T = See descriptions above in this table, stage IV, Any T, N2, M0.
NX = Regional lymph nodes cannot be assessed.
N0 = No regional lymph node metastasis.
N1 = Single regional lymph node metastasis in the inguinal region or true pelvis (perivesical, obturator, internal [hypogastric] and external iliac), or presacral lymph node.
N2 = Multiple regional lymph node metastasis in the inguinal region or true pelvis (perivesical, obturator, internal [hypogastric] and external iliac), or presacral lymph node.
M1 = Distant metastasis.
References
  1. Urethra. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. Springer; 2017, pp. 767–76.
  2. Grigsby PW, Corn BW: Localized urethral tumors in women: indications for conservative versus exenterative therapies. J Urol 147 (6): 1516-20, 1992. [PUBMED Abstract]
  3. Ryu J, Kim B: MR imaging of the male and female urethra. Radiographics 21 (5): 1169-85, 2001 Sep-Oct. [PUBMED Abstract]
  4. Pavlica P, Barozzi L, Menchi I: Imaging of male urethra. Eur Radiol 13 (7): 1583-96, 2003. [PUBMED Abstract]
  5. Mark JR, Hurwitz M, Gomella LG: Cancer of the urethra and penis. In: DeVita VT Jr, Lawrence TS, Rosenberg SA, et al., eds.: DeVita, Hellman, and Rosenberg’s Cancer: Principles & Practice of Oncology. 11th ed. Wolters Kluwer, 2019, pp 1136-44.
  6. Erckert M, Stenzl A, Falk M, et al.: Incidence of urethral tumor involvement in 910 men with bladder cancer. World J Urol 14 (1): 3-8, 1996. [PUBMED Abstract]

Treatment Option Overview for Urethral Cancer

Information about the treatment of urethral cancer and the outcomes of therapy is derived from retrospective, single-center case series and represents a very low Level of evidence C3. Most of this information comes from the small numbers of cases accumulated over many decades at major academic centers. Therefore, the treatment in these reports is usually not standardized and spans eras of shifting supportive care practices. Because urethral cancer is rare, its treatment may also reflect extrapolation from the management of other urothelial malignancies, such as bladder cancer in the case of transitional cancers and anal cancer in the case of squamous cell carcinomas.

Surgery

Surgery is the mainstay of therapy for urethral cancers in both women and men.[Level of evidence C3] The surgical approach depends on tumor stage and anatomic location, and tumor grade plays a less important role in treatment decisions.[1,2] Although the traditional recommendation has been to achieve a 2-cm tumor-free margin, the optimal surgical margin has not been rigorously studied and is not well defined. The role of lymph node dissection is not clear in the absence of clinical involvement, and the role of prophylactic dissection is controversial.[2] Radiation therapy and/or chemotherapy may be added in some cases in patients with extensive disease or in an attempt at organ preservation. However, there are no clear guidelines for patient selection, and the low level of evidence precludes confident conclusions about their incremental benefit.[2,3]

Ablative techniques, such as transurethral resection, electroresection and fulguration, or laser vaporization-coagulation, are used to preserve organ function in cases of superficial anterior tumors, although the supporting literature is scant.[2]

Radiation Therapy

External-beam radiation therapy, brachytherapy, or a combination is sometimes used as the primary therapy for early-stage proximal urethral cancers, particularly in women.[Level of evidence C3] Brachytherapy may be delivered with low-dose-rate iridium Ir 192 sources using a template or urethral catheter. Definitive radiation is also sometimes used for advanced-stage tumors, but because monotherapy of large tumors has shown poor tumor control, it is more frequently incorporated into combined modality therapy after surgery or with chemotherapy.[4] There are no head-to-head comparisons of these various approaches, and patient selection may explain differences in outcomes among the regimens.[Level of evidence C3]

The most commonly used tumor doses are in the range of 60 Gy to 70 Gy. Severe complication rates for definitive radiation therapy are about 16% to 20% and include fistula development, especially for large tumors invading the vagina, bladder, or rectum. Urethral strictures also occur in the setting of urethral-sparing treatment. Toxicity rates increase at doses greater than 65 Gy to 70 Gy. Intensity-modulated radiation therapy has become more common in an attempt to decrease local morbidity of the radiation.[4]

Chemotherapy

The literature on chemotherapy for urethral carcinoma is anecdotal in nature and restricted to retrospective, single-center case series or case reports.[5][Level of evidence C3] A wide variety of agents used alone or in combination have been reported over the years, and their use has largely been extrapolated from experience with other urinary tract tumors.

For squamous cell cancers, agents that have been used in penile cancer or anal carcinoma include the following:[3,5]

  • Cisplatin.
  • Fluorouracil.
  • Bleomycin.
  • Methotrexate.
  • Irinotecan.
  • Gemcitabine.
  • Paclitaxel.
  • Docetaxel.
  • Mitomycin.

Chemotherapy for transitional cell urethral tumors is extrapolated from experience with transitional cell bladder tumors and, therefore, usually contains the following:[1,47]

  • Methotrexate, vinblastine, doxorubicin, and cisplatin.
  • Paclitaxel.
  • Carboplatin.
  • Ifosfamide, with occasional complete responses.

Chemotherapy has been used alone for metastatic disease or in combination with radiation therapy and/or surgery for locally advanced urethral cancer. It may be used in the neoadjuvant setting with radiation therapy in an attempt to increase the resectability rate or to preserve organs.[3] However, the impact of any of these regimens on survival is not known for any stage or setting.

Fluorouracil dosing

The DPYD gene encodes an enzyme that catabolizes pyrimidines and fluoropyrimidines, like capecitabine and fluorouracil. An estimated 1% to 2% of the population has germline pathogenic variants in DPYD, which lead to reduced DPD protein function and an accumulation of pyrimidines and fluoropyrimidines in the body.[8,9] Patients with the DPYD*2A variant who receive fluoropyrimidines may experience severe, life-threatening toxicities that are sometimes fatal. Many other DPYD variants have been identified, with a range of clinical effects.[810] Fluoropyrimidine avoidance or a dose reduction of 50% may be recommended based on the patient’s DPYD genotype and number of functioning DPYD alleles.[1113] DPYD genetic testing costs less than $200, but insurance coverage varies due to a lack of national guidelines.[14] In addition, testing may delay therapy by 2 weeks, which would not be advisable in urgent situations. This controversial issue requires further evaluation.[15]

References
  1. Mark JR, Hurwitz M, Gomella LG: Cancer of the urethra and penis. In: DeVita VT Jr, Lawrence TS, Rosenberg SA, et al., eds.: DeVita, Hellman, and Rosenberg’s Cancer: Principles & Practice of Oncology. 11th ed. Wolters Kluwer, 2019, pp 1136-44.
  2. Karnes RJ, Breau RH, Lightner DJ: Surgery for urethral cancer. Urol Clin North Am 37 (3): 445-57, 2010. [PUBMED Abstract]
  3. Cohen MS, Triaca V, Billmeyer B, et al.: Coordinated chemoradiation therapy with genital preservation for the treatment of primary invasive carcinoma of the male urethra. J Urol 179 (2): 536-41; discussion 541, 2008. [PUBMED Abstract]
  4. Koontz BF, Lee WR: Carcinoma of the urethra: radiation oncology. Urol Clin North Am 37 (3): 459-66, 2010. [PUBMED Abstract]
  5. Trabulsi EJ, Hoffman-Censits J: Chemotherapy for penile and urethral carcinoma. Urol Clin North Am 37 (3): 467-74, 2010. [PUBMED Abstract]
  6. VanderMolen LA, Sheehy PF, Dillman RO: Successful treatment of transitional cell carcinoma of the urethra with chemotherapy. Cancer Invest 20 (2): 206-7, 2002. [PUBMED Abstract]
  7. Lin CC, Hsu CH, Huang CY, et al.: Phase II trial of weekly paclitaxel, cisplatin plus infusional high dose 5-fluorouracil and leucovorin for metastatic urothelial carcinoma. J Urol 177 (1): 84-9; discussion 89, 2007. [PUBMED Abstract]
  8. Sharma BB, Rai K, Blunt H, et al.: Pathogenic DPYD Variants and Treatment-Related Mortality in Patients Receiving Fluoropyrimidine Chemotherapy: A Systematic Review and Meta-Analysis. Oncologist 26 (12): 1008-1016, 2021. [PUBMED Abstract]
  9. Lam SW, Guchelaar HJ, Boven E: The role of pharmacogenetics in capecitabine efficacy and toxicity. Cancer Treat Rev 50: 9-22, 2016. [PUBMED Abstract]
  10. Shakeel F, Fang F, Kwon JW, et al.: Patients carrying DPYD variant alleles have increased risk of severe toxicity and related treatment modifications during fluoropyrimidine chemotherapy. Pharmacogenomics 22 (3): 145-155, 2021. [PUBMED Abstract]
  11. Amstutz U, Henricks LM, Offer SM, et al.: Clinical Pharmacogenetics Implementation Consortium (CPIC) Guideline for Dihydropyrimidine Dehydrogenase Genotype and Fluoropyrimidine Dosing: 2017 Update. Clin Pharmacol Ther 103 (2): 210-216, 2018. [PUBMED Abstract]
  12. Henricks LM, Lunenburg CATC, de Man FM, et al.: DPYD genotype-guided dose individualisation of fluoropyrimidine therapy in patients with cancer: a prospective safety analysis. Lancet Oncol 19 (11): 1459-1467, 2018. [PUBMED Abstract]
  13. Lau-Min KS, Varughese LA, Nelson MN, et al.: Preemptive pharmacogenetic testing to guide chemotherapy dosing in patients with gastrointestinal malignancies: a qualitative study of barriers to implementation. BMC Cancer 22 (1): 47, 2022. [PUBMED Abstract]
  14. Brooks GA, Tapp S, Daly AT, et al.: Cost-effectiveness of DPYD Genotyping Prior to Fluoropyrimidine-based Adjuvant Chemotherapy for Colon Cancer. Clin Colorectal Cancer 21 (3): e189-e195, 2022. [PUBMED Abstract]
  15. Baker SD, Bates SE, Brooks GA, et al.: DPYD Testing: Time to Put Patient Safety First. J Clin Oncol 41 (15): 2701-2705, 2023. [PUBMED Abstract]

Treatment of Distal Urethral Cancer

Treatment Options for Female Distal Urethral Cancer

If the malignancy is at or just within the meatus and superficial parameters (stage 0/Tis, Ta), open excision or electroresection and fulguration may be possible. Tumor destruction using neodymium:yttrium-aluminum-garnet (Nd:YAG) or CO2 laser vaporization-coagulation represents an alternative option. For large lesions (T1) and more invasive lesions (T2), brachytherapy or a combination of brachytherapy and external-beam radiation therapy (EBRT) are alternatives to surgical resection of the distal third of the urethra. Patients with T3 distal urethral lesions or lesions that recur after treatment with local excision or radiation therapy require anterior exenteration and urinary diversion.

If inguinal lymph nodes are palpable, frozen section confirmation of a tumor should be obtained. If positive for malignancy, ipsilateral lymph node dissection is indicated. If no inguinal adenopathy exists, lymph node dissection is not generally performed, and the nodes are monitored clinically.

Treatment options for female distal urethral cancer include the following:

  1. Open excision and organ-sparing conservative surgical therapy.[1]
  2. Ablative techniques, such as transurethral resection, electroresection and fulguration, or laser vaporization-coagulation (Tis, Ta, T1 lesions).[2,3]
  3. EBRT, brachytherapy, or a combination of the two (T1, T2 lesions).[4]
  4. Anterior exenteration with or without preoperative radiation and diversion (T3 lesions or recurrent lesions).[2,3]

The level of evidence for these treatment options is Level of evidence C3.

Treatment Options for Male Distal Urethral Cancer

If the malignancy is in the pendulous urethra and is superficial, there is potential for long-term disease-free survival. In the rare cases that involve mucosa only (Tis, Ta), resection and fulguration may be used. For infiltrating lesions in the fossa navicularis, amputation of the glans penis may be adequate treatment. For lesions involving more proximal portions of the distal urethra, excision of the involved segment of the urethra, preserving the penile corpora, may be feasible for superficial tumors. Penile amputation is used for infiltrating lesions. Traditionally, a 2-cm margin proximal to the tumor is used, but the optimal margin has not been well studied. Local recurrences after amputation are rare.

The role of radiation therapy in the treatment of anterior urethral carcinoma in men is not well defined. Some anterior urethral cancers have been cured with radiation alone or a combination of chemotherapy and radiation therapy.[4,5]

If inguinal lymph nodes are palpable, ipsilateral node dissection is indicated after frozen section confirmation of a tumor because a cure is still achievable with limited regional nodal metastases. If no inguinal adenopathy exists, lymph node dissection is not generally performed, and the nodes are monitored clinically.

Treatment options for male distal urethral cancer include the following:

  1. Open-excision and organ-sparing conservative surgery.[1,3]
  2. Ablative techniques, such as transurethral resection, electroresection and fulguration, or laser vaporization-coagulation (Tis, Ta, T1 lesions).[2,3]
  3. Amputation of the penis (T1, T2, T3 lesions).
  4. Radiation (T1, T2, T3 lesions, if amputation is refused).[4]
  5. Combined chemotherapy and radiation therapy.[5]

The level of evidence for these treatment options is Level of evidence C3.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Davis JW, Schellhammer PF, Schlossberg SM: Conservative surgical therapy for penile and urethral carcinoma. Urology 53 (2): 386-92, 1999. [PUBMED Abstract]
  2. Mark JR, Hurwitz M, Gomella LG: Cancer of the urethra and penis. In: DeVita VT Jr, Lawrence TS, Rosenberg SA, et al., eds.: DeVita, Hellman, and Rosenberg’s Cancer: Principles & Practice of Oncology. 11th ed. Wolters Kluwer, 2019, pp 1136-44.
  3. Karnes RJ, Breau RH, Lightner DJ: Surgery for urethral cancer. Urol Clin North Am 37 (3): 445-57, 2010. [PUBMED Abstract]
  4. Koontz BF, Lee WR: Carcinoma of the urethra: radiation oncology. Urol Clin North Am 37 (3): 459-66, 2010. [PUBMED Abstract]
  5. Cohen MS, Triaca V, Billmeyer B, et al.: Coordinated chemoradiation therapy with genital preservation for the treatment of primary invasive carcinoma of the male urethra. J Urol 179 (2): 536-41; discussion 541, 2008. [PUBMED Abstract]

Treatment of Proximal Urethral Cancer

Treatment Options for Female Proximal Urethral Cancer

Lesions of the proximal urethra or the entire length of the urethra are usually associated with invasion and a high incidence of pelvic nodal metastases. The prospects for cure are limited except in the case of small tumors. The best results have been achieved with exenterative surgery and urinary diversion, with 5-year survival rates ranging from 10% to 20%.

In an effort to shrink tumor margins, increase the resectability rate of gross tumor, and decrease local recurrence, adjunctive preoperative radiation therapy is a reasonable option. Pelvic lymphadenectomy is performed concomitantly. Ipsilateral inguinal lymph node dissection is indicated only if biopsy specimens of ipsilateral palpable adenopathy are positive on a frozen section. For tumors that do not exceed 2 cm in greatest dimension, radiation alone, nonexenterative surgery alone, or a combination of the two may be sufficient to provide an excellent outcome.

It is reasonable to consider removal of part of the pubic symphysis and the inferior pubic rami to maximize the surgical margin and reduce local recurrence. The perineal closure and vaginal reconstruction can be accomplished with the use of myocutaneous flaps.

The prognosis of female urethral cancer is related to the size of the lesion at presentation. For lesions smaller than 2 cm in diameter, a 5-year survival rate of 60% can be anticipated. For lesions larger than 4 cm in diameter, the 5-year survival rate falls to 13%.

Treatment options for female proximal urethral cancer include the following:

  1. Preoperative radiation followed by anterior exenteration and urinary diversion with bilateral pelvic lymph node dissection, with or without inguinal lymph node dissection.[1]
  2. For tumors that do not exceed 2 cm in greatest dimension, radiation alone, nonexenterative surgery alone, or a combination of the two may be sufficient to provide an excellent outcome.[1,2]

The level of evidence for these treatment options is Level of evidence C3.

Treatment Options for Male Proximal Urethral Cancer

Lesions of the bulbomembranous urethra require radical cystoprostatectomy and en bloc penectomy to achieve adequate margins of resection, minimize local recurrence, and achieve long-term, disease-free survival. Pelvic lymphadenectomy is also performed because of the high incidence of positive lymph nodes and the limited added morbidity.

Despite extensive surgery, local recurrence is common, and this event is invariably associated with eventual death from the disease. Five-year survival rates are only 15% to 20%. In an effort to shrink tumor margins, the use of preoperative adjunctive radiation therapy may be considered. In an effort to increase the surgical margins of dissection, resection of the inferior pubic rami and the lower portion of the pubic symphysis has been used. Urinary diversion is required.[3]

Ipsilateral inguinal lymph node dissection is indicated if palpable ipsilateral inguinal adenopathy is found on physical examination and confirmed to be neoplasm by frozen section.

Treatment options for male proximal urethral cancer include the following:

  1. Preoperative radiation or combined chemotherapy and radiation therapy followed by cystoprostatectomy, urinary diversion, and penectomy with bilateral pelvic lymph node dissection, with or without inguinal lymph node dissection.[4]

The level of evidence for these treatment options is Level of evidence C3.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Koontz BF, Lee WR: Carcinoma of the urethra: radiation oncology. Urol Clin North Am 37 (3): 459-66, 2010. [PUBMED Abstract]
  2. Grigsby PW, Corn BW: Localized urethral tumors in women: indications for conservative versus exenterative therapies. J Urol 147 (6): 1516-20, 1992. [PUBMED Abstract]
  3. Su LM, Smith JA Jr.: Laparoscopic and robotic-assisted laparoscopic radical prostatectomy and pelvic lymphadenectomy. In: Wein AJ, Kavoussi LR, Novick AC, et al.: Campbell-Walsh Urology. 10th ed. Elsevier Saunders, 2012, pp 2830-2849.
  4. Karnes RJ, Breau RH, Lightner DJ: Surgery for urethral cancer. Urol Clin North Am 37 (3): 445-57, 2010. [PUBMED Abstract]

Treatment of Urethral Cancer Associated With Invasive Bladder Cancer

Approximately 10% (range, 4%–17%) of patients who undergo cystectomy for bladder cancer can be expected to have or to later develop clinical neoplasm of the urethra distal to the urogenital diaphragm. Factors associated with the risk of urethral recurrence after cystectomy include the following:[1,2]

  • Tumor multiplicity.
  • Papillary pattern.
  • Carcinoma in situ.
  • Tumor location at the bladder neck.
  • Prostatic urethral mucosal or stromal involvement.

The benefits of urethrectomy at the time of cystectomy need to be weighed against the morbidity factors, which include added operating time, hemorrhage, and the potential for perineal hernia. Tumors found incidentally on pathological examination are much more likely to be superficial or in situ in contrast to those that present with clinical symptoms at a later date, when the likelihood of invasion within the corporal bodies is high. The former lesions are often curable, and the latter are only rarely so. Indications for urethrectomy in continuity with cystoprostatectomy are the following:

  • Visible tumor in the urethra.
  • Positive swab cytology of the urethra.
  • Positive margins of the membranous urethra on frozen section taken at the time of cystoprostatectomy.
  • Multiple in situ bladder tumors that extend onto the bladder neck and proximal prostatic urethra.

If the urethra is not removed at the time of cystectomy, follow-up includes periodic cytologic evaluation of saline urethral washings.[2]

Treatment options for urethral cancer associated with invasive bladder cancer include the following:

  1. In continuity cystourethrectomy.
  2. Monitor urethral cytology and delayed urethrectomy, if necessary.

The level of evidence for these treatment options is Level of evidence C3.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Mark JR, Hurwitz M, Gomella LG: Cancer of the urethra and penis. In: DeVita VT Jr, Lawrence TS, Rosenberg SA, et al., eds.: DeVita, Hellman, and Rosenberg’s Cancer: Principles & Practice of Oncology. 11th ed. Wolters Kluwer, 2019, pp 1136-44.
  2. Sherwood JB, Sagalowsky AI: The diagnosis and treatment of urethral recurrence after radical cystectomy. Urol Oncol 24 (4): 356-61, 2006 Jul-Aug. [PUBMED Abstract]

Treatment of Metastatic or Recurrent Urethral Cancer

Local recurrences of urethral cancer may be amenable to local modality therapy with radiation or surgery, with or without chemotherapy. For more information, see the Treatment Option Overview for Urethral Cancer section. Metastatic disease may be treated with regimens in common use for other urothelial transitional cell or squamous cell carcinomas, or anal carcinomas, depending on the histology.[13]

Treatment options for metastatic or recurrent urethral cancer include the following:

  1. Surgical excision of locally recurrent urethral cancer after radiation therapy should be considered, if feasible.
  2. Combination radiation therapy and wider surgical resection should be considered for locally recurrent urethral cancer after surgery alone.
  3. Clinical trials using chemotherapy should be considered for metastatic urethral cancer. Transitional cell cancer of the urethra may respond favorably to the same chemotherapy regimens used for advanced transitional cell cancer of the bladder.[14]

The level of evidence for these treatment options is Level of evidence C3.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Mark JR, Hurwitz M, Gomella LG: Cancer of the urethra and penis. In: DeVita VT Jr, Lawrence TS, Rosenberg SA, et al., eds.: DeVita, Hellman, and Rosenberg’s Cancer: Principles & Practice of Oncology. 11th ed. Wolters Kluwer, 2019, pp 1136-44.
  2. Trabulsi EJ, Hoffman-Censits J: Chemotherapy for penile and urethral carcinoma. Urol Clin North Am 37 (3): 467-74, 2010. [PUBMED Abstract]
  3. Lin CC, Hsu CH, Huang CY, et al.: Phase II trial of weekly paclitaxel, cisplatin plus infusional high dose 5-fluorouracil and leucovorin for metastatic urothelial carcinoma. J Urol 177 (1): 84-9; discussion 89, 2007. [PUBMED Abstract]
  4. VanderMolen LA, Sheehy PF, Dillman RO: Successful treatment of transitional cell carcinoma of the urethra with chemotherapy. Cancer Invest 20 (2): 206-7, 2002. [PUBMED Abstract]

Latest Updates to This Summary (07/19/2024)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Treatment Option Overview for Urethral Cancer

Added Fluorouracil dosing as a new subsection.

This summary is written and maintained by the PDQ Adult Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® Cancer Information for Health Professionals pages.

About This PDQ Summary

Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of urethral cancer. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.

Reviewers and Updates

This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

Board members review recently published articles each month to determine whether an article should:

  • be discussed at a meeting,
  • be cited with text, or
  • replace or update an existing article that is already cited.

Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.

The lead reviewers for Urethral Cancer Treatment are:

  • Juskaran S. Chadha, DO (Moffitt Cancer Center)
  • Jad Chahoud, MD, MPH (Moffitt Cancer Center)
  • Timothy Gilligan, MD (Cleveland Clinic Taussig Cancer Institute)

Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website’s Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

Levels of Evidence

Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.

Permission to Use This Summary

PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”

The preferred citation for this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Urethral Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/urethral/hp/urethral-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389356]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in Visuals Online, a collection of over 2,000 scientific images.

Disclaimer

Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s Email Us.

Urethral Cancer—Health Professional Version

Urethral Cancer—Health Professional Version

Treatment

PDQ Treatment Information for Health Professionals

More information

Causes & Prevention

NCI does not have PDQ evidence-based information about prevention of urethral cancer.

Screening

NCI does not have PDQ evidence-based information about screening for urethral cancer.

Supportive & Palliative Care

We offer evidence-based supportive and palliative care information for health professionals on the assessment and management of cancer-related symptoms and conditions.

Cancer Pain Nausea and Vomiting Nutrition in Cancer Care Transition to End-of-Life Care Last Days of Life View all Supportive and Palliative Care Summaries

Urethral Cancer—Patient Version

Urethral Cancer—Patient Version

Overview

Urethral cancer is rare and is more common in men than in women. Urethral cancer can metastasize (spread) quickly to tissues around the urethra and has often spread to nearby lymph nodes by the time it is diagnosed. Explore the links on this page to learn more about urethral cancer treatment and clinical trials.

Treatment

PDQ Treatment Information for Patients

More information

Causes & Prevention

NCI does not have PDQ evidence-based information about prevention of urethral cancer.

Screening

NCI does not have PDQ evidence-based information about screening for urethral cancer.

Coping with Cancer

The information in this section is meant to help you cope with the many issues and concerns that occur when you have cancer.

Emotions and Cancer Adjusting to Cancer Support for Caregivers Survivorship Advanced Cancer Managing Cancer Care

Small Intestine Cancer Treatment (PDQ®)–Patient Version

Small Intestine Cancer Treatment (PDQ®)–Patient Version

General Information About Small Intestine Cancer

Key Points

  • Small intestine cancer is a rare disease in which malignant (cancer) cells form in the tissues of the small intestine.
  • There are five types of small intestine cancer.
  • Diet and health history can affect the risk of developing small intestine cancer.
  • Signs and symptoms of small intestine cancer include unexplained weight loss and abdominal pain.
  • Tests that examine the small intestine are used to diagnose and stage small intestine cancer.
  • Certain factors affect prognosis (chance of recovery) and treatment options.

Small intestine cancer is a rare disease in which malignant (cancer) cells form in the tissues of the small intestine.

The small intestine is part of the body’s digestive system, which also includes the esophagus, stomach, and large intestine. The digestive system removes and processes nutrients (vitamins, minerals, carbohydrates, fats, proteins, and water) from foods and helps pass waste material out of the body. The small intestine is a long tube that connects the stomach to the large intestine. It folds many times to fit inside the abdomen.

EnlargeDrawing of the small intestine showing the duodenum, jejunum, and ileum. Also shown are the stomach, appendix, large intestine, and rectum.
The small intestine is a long tube-like organ that connects the stomach to the large intestine. The small intestine includes the duodenum, jejunum, and ileum.

There are five types of small intestine cancer.

The types of cancer found in the small intestine are adenocarcinoma, sarcoma, neuroendocrine tumors, gastrointestinal stromal tumor, and lymphoma. This summary discusses adenocarcinoma and leiomyosarcoma (a type of sarcoma).

Adenocarcinoma starts in glandular cells in the lining of the small intestine and is the most common type of small intestine cancer. Most of these tumors occur in the part of the small intestine near the stomach. They may grow and block the intestine.

Leiomyosarcoma starts in the smooth muscle cells of the small intestine. Most of these tumors occur in the part of the small intestine near the large intestine.

For more information on small intestine cancer, see the following:

Diet and health history can affect the risk of developing small intestine cancer.

Anything that increases a person’s chance of getting a disease is called a risk factor. Not every person with one or more of these risk factors will develop small intestine cancer, and it will develop in some people who don’t have any known risk factors. Talk with your doctor if you think you may be at risk. Risk factors for small intestine cancer include the following:

Signs and symptoms of small intestine cancer include unexplained weight loss and abdominal pain.

These and other signs and symptoms may be caused by small intestine cancer or by other conditions. Check with your doctor if you have any of the following:

  • Pain or cramps in the middle of the abdomen.
  • Weight loss with no known reason.
  • A lump in the abdomen.
  • Blood in the stool.

Tests that examine the small intestine are used to diagnose and stage small intestine cancer.

Procedures that make pictures of the small intestine and the area around it help diagnose small intestine cancer and show how far the cancer has spread. The process used to find out if cancer cells have spread within and around the small intestine is called staging.

In order to plan treatment, it is important to know the type of small intestine cancer and whether the tumor can be removed by surgery. Tests and procedures to detect, diagnose, and stage small intestine cancer are usually done at the same time. In addition to asking about your personal and family health history and doing a physical exam, your doctor may perform the following tests and procedures:

  • Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
  • Liver function tests: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by the liver. A higher than normal amount of a substance can be a sign of liver disease that may be caused by small intestine cancer.
  • Endoscopy: A procedure to look at organs and tissues inside the body to check for abnormal areas. There are different types of endoscopy:
    • Upper endoscopy: A procedure to look at the inside of the esophagus, stomach, and duodenum (first part of the small intestine, near the stomach). An endoscope is inserted through the mouth and into the esophagus, stomach, and duodenum. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
    • Capsule endoscopy: A procedure to look at the inside of the small intestine. A capsule that is about the size of a large pill and contains a light and a tiny wireless camera is swallowed by the patient. The capsule travels through the digestive tract, including the small intestine, and sends many pictures of the inside of the digestive tract to a recorder that is worn around the waist or over the shoulder. The pictures are sent from the recorder to a computer and viewed by the doctor who checks for signs of cancer. The capsule passes out of the body during a bowel movement.
    • Double balloon endoscopy: A procedure to look at the inside of the small intestine. A special instrument made up of two tubes (one inside the other) is inserted through the mouth or rectum and into the small intestine. The inside tube (an endoscope with a light and lens for viewing) is moved through part of the small intestine and a balloon at the end of it is inflated to keep the endoscope in place. Next, the outer tube is moved through the small intestine to reach the end of the endoscope, and a balloon at the end of the outer tube is inflated to keep it in place. Then, the balloon at the end of the endoscope is deflated and the endoscope is moved through the next part of the small intestine. These steps are repeated many times as the tubes move through the small intestine. The doctor is able to see the inside of the small intestine through the endoscope and use a tool to remove samples of abnormal tissue. The tissue samples are checked under a microscope for signs of cancer. This procedure may be done if the results of a capsule endoscopy are abnormal. This procedure is also called double balloon enteroscopy.
  • Laparotomy: A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs or lymph nodes are removed or tissue samples are taken and checked under a microscope for signs of disease.
  • Biopsy: The removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer. This may be done during an endoscopy or laparotomy. The sample is checked by a pathologist to see if it contains cancer cells.
  • Upper GI series with small bowel follow-through: A series of x-rays of the esophagus, stomach, and small bowel. The patient drinks a liquid that contains barium (a silver-white metallic compound). The liquid coats the esophagus, stomach, and small bowel. X-rays are taken at different times as the barium travels through the upper GI tract and small bowel.
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis and treatment options depend on the following:

  • The type of small intestine cancer.
  • Whether the cancer is in the inner lining of the small intestine only or has spread into or beyond the wall of the small intestine.
  • Whether the cancer has spread to other places in the body, such as the lymph nodes, liver, or peritoneum (tissue that lines the wall of the abdomen and covers most of the organs in the abdomen).
  • Whether the cancer can be completely removed by surgery.
  • Whether the cancer is newly diagnosed or has recurred.

Stages of Small Intestine Cancer

Key Points

  • Tests and procedures to stage small intestine cancer are usually done at the same time as diagnosis.
  • There are three ways that cancer spreads in the body.
  • Cancer may spread from where it began to other parts of the body.
  • Small intestine cancer is grouped according to whether or not the tumor can be completely removed by surgery.
  • Small intestine cancer can recur (come back) after it has been treated.

Tests and procedures to stage small intestine cancer are usually done at the same time as diagnosis.

Staging is used to find out how far the cancer has spread, but treatment decisions are not based on stage. See the General Information section for a description of tests and procedures used to detect, diagnose, and stage small intestine cancer.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

  • Tissue. The cancer spreads from where it began by growing into nearby areas.
  • Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
  • Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

  • Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
  • Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if small intestine cancer spreads to the liver, the cancer cells in the liver are actually small intestine cancer cells. The disease is metastatic small intestine cancer, not liver cancer.

Many cancer deaths are caused when cancer moves from the original tumor and spreads to other tissues and organs. This is called metastatic cancer. This animation shows how cancer cells travel from the place in the body where they first formed to other parts of the body.

Small intestine cancer is grouped according to whether or not the tumor can be completely removed by surgery.

Treatment depends on whether the tumor can be removed by surgery and if the cancer is being treated as a primary tumor or is metastatic cancer.

Small intestine cancer can recur (come back) after it has been treated.

The cancer may come back in the small intestine or in other parts of the body.

Treatment Option Overview

Key Points

  • There are different types of treatment for patients with small intestine cancer.
  • The following types of treatment are used:
    • Surgery
    • Radiation therapy
    • Chemotherapy
  • New types of treatment are being tested in clinical trials.
    • Immunotherapy
    • Radiation therapy with radiosensitizers
  • Treatment for small intestine cancer may cause side effects.
  • Patients may want to think about taking part in a clinical trial.
  • Patients can enter clinical trials before, during, or after starting their cancer treatment.
  • Follow-up tests may be needed.

There are different types of treatment for patients with small intestine cancer.

Different types of treatments are available for patients with small intestine cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

The following types of treatment are used:

Surgery

Surgery is the most common treatment of small intestine cancer. One of the following types of surgery may be done:

  • Resection: Surgery to remove part or all of an organ that contains cancer. The resection may include the small intestine and nearby organs (if the cancer has spread). The doctor may remove the section of the small intestine that contains cancer and perform an anastomosis (joining the cut ends of the intestine together). The doctor will usually remove lymph nodes near the small intestine and examine them under a microscope to see whether they contain cancer.
  • Bypass: Surgery to allow food in the small intestine to go around (bypass) a tumor that is blocking the intestine but cannot be removed.

After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer.

External radiation therapy is used to treat small intestine cancer.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy).

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.

Immunotherapy

Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer.

Radiation therapy with radiosensitizers

Radiosensitizers are drugs that make tumor cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers may kill more tumor cells.

Treatment for small intestine cancer may cause side effects.

For information about side effects caused by treatment for cancer, visit our Side Effects page.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today’s standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

Follow-up tests may be needed.

As you go through treatment, you will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back).

Treatment of Small Intestine Adenocarcinoma

For information about the treatments listed below, see the Treatment Option Overview section.

When possible, treatment of small intestine adenocarcinoma will be surgery to remove the tumor and some of the normal tissue around it.

Treatment of small intestine adenocarcinoma that cannot be removed by surgery may include the following:

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Small Intestine Leiomyosarcoma

For information about the treatments listed below, see the Treatment Option Overview section.

When possible, treatment of small intestine leiomyosarcoma will be surgery to remove the tumor and some of the normal tissue around it.

Treatment of small intestine leiomyosarcoma that cannot be removed by surgery may include the following:

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Recurrent Small Intestine Cancer

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of recurrent small intestine cancer that has spread to other parts of the body is usually a clinical trial of new anticancer drugs or immunotherapy.

Treatment of locally recurrent small intestine cancer may include the following:

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

To Learn More About Small Intestine Cancer

About This PDQ Summary

About PDQ

Physician Data Query (PDQ) is the National Cancer Institute’s (NCI’s) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.

PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of small intestine cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

Reviewers and Updates

Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary (“Updated”) is the date of the most recent change.

The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.

Clinical Trial Information

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become “standard.” Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Clinical trials can be found online at NCI’s website. For more information, call the Cancer Information Service (CIS), NCI’s contact center, at 1-800-4-CANCER (1-800-422-6237).

Permission to Use This Summary

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Small Intestine Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/small-intestine/patient/small-intestine-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389461]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.

Small Intestine Cancer Treatment (PDQ®)–Health Professional Version

Small Intestine Cancer Treatment (PDQ®)–Health Professional Version

General Information About Small Intestine Cancer

Incidence and Mortality

Estimated new cases and deaths from small intestine cancer in the United States in 2025:[1]

  • New cases: 13,920.
  • Deaths: 2,060.

Small intestine cancer types include adenocarcinoma, sarcoma, neuroendocrine tumors, gastrointestinal stromal tumors, and lymphoma. Small intestine cancer accounts for 3.8% of all digestive system malignancies.[1,2]

Follow-Up and Survivorship

As in other gastrointestinal malignancies, the predominant modality of treatment is surgery when resection is possible, and cure relates to the ability to completely resect the cancer.

Neuroendocrine tumors of the small intestine are covered in another summary as a separate cancer entity. For more information, see Gastrointestinal Neuroendocrine Tumors Treatment.

References
  1. American Cancer Society: Cancer Facts and Figures 2025. American Cancer Society, 2025. Available online. Last accessed January 16, 2025.
  2. National Cancer Institute: SEER Cancer Stat Facts: Small Intestine Cancer. Bethesda, Md: National Cancer Institute. Available online. Last accessed March 6, 2025.

Cellular Classification of Small Intestine Cancer

The following tumors occur in the small intestine:

Approximately 25% to 50% of the primary malignant tumors in the small intestine are adenocarcinomas, and most occur in the duodenum.[1] Small intestine carcinomas may occur synchronously or metachronously at multiple sites.

Leiomyosarcomas occur most often in the ileum.

About 20% of malignant lesions of the small intestine are neuroendocrine tumors, which occur more frequently in the ileum than in the duodenum or jejunum and may be multiple.

It is uncommon to find malignant lymphoma as a solitary small intestine lesion.

References
  1. Small Intestine. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. Springer; 2017, pp. 221–34.

Stage Information for Small Intestine Cancer

The treatment sections of this summary are organized according to histopathological type rather than stage.

AJCC Stage Groupings and TNM Definitions

The American Joint Committee on Cancer (AJCC) has designated staging by TNM (tumor, node, metastasis) classification to define small intestine cancer. This staging classification applies only to adenocarcinomas arising in the nonampullary duodenum and small intestine. Nonadenocarcinomas arising in the small intestine should have a TNM assigned but are not assigned a stage classification.[1]

Table 1. Definitions of Primary Tumor (T)a
T Category T Criteria
aReprinted with permission from AJCC: Small Intestine. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 221–34.
bFor T3 tumors, the nonperitonealized perimuscular tissue is, for the jejunum and ileum, part of the mesentery and, for the duodenum in areas where serosa is lacking, part of the interface with the pancreas.
TX Primary tumor cannot be assessed.
T0 No evidence of primary tumor.
Tis High-grade dysplasia/carcinoma in situ.
T1 Tumor invades the lamina propria or submucosa.
−T1a Tumor invades the lamina propria.
−T1b Tumor invades the submucosa.
T2 Tumor invades the muscularis propria.
T3 Tumor invades through the muscularis propria into the subserosa, or extends into nonperitonealized perimuscular tissue (mesentery or retroperitoneum) without serosal penetration.b
T4 Tumor perforates the visceral peritoneum or directly invades other organs or structures (e.g., other loops of small intestine, mesentery of adjacent loops of bowel, and abdominal wall by way of serosa; for duodenum only, invasion of pancreas or bile duct).
Table 2. Definitions of Regional Lymph Node (N)a
N Category N Criteria
aReprinted with permission from AJCC: Small Intestine. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 221–34.
NX Regional lymph nodes cannot be assessed.
N0 No regional lymph node metastasis.
N1 Metastasis in one or two regional lymph nodes.
N2 Metastasis in three or more regional lymph nodes.
Table 3. Definitions of Distant Metastasis (M)a
M Category M Criteria
aReprinted with permission from AJCC: Small Intestine. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 221–34.
M0 No distant metastasis.
M1 Distant metastasis present.
Table 4. Prognostic Stage Groups for Adenocarcinomaa
Stage T N M
T = primary tumor; N = regional lymph node; M = distant metastasis.
aReprinted with permission from AJCC: Small Intestine. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 221–34.
0 Tis N0 M0
I T1−2 N0 M0
IIA T3 N0 M0
IIB T4 N0 M0
IIIA Any T N1 M0
IIIB Any T N2 M0
IV Any T Any N M1
References
  1. Small Intestine. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. Springer; 2017, pp. 221–34.

Treatment of Small Intestine Adenocarcinoma

Treatment options:

  1. For resectable primary disease:
    • Radical surgical resection.[1,2]
  2. For unresectable primary disease:
    • Surgical bypass of obstructing lesion.
    • Palliative radiation therapy.
    • Clinical trials evaluating methods to improve local control, such as the use of radiation therapy with radiosensitizers with or without systemic chemotherapy.
  3. For unresectable metastatic disease:
    • Clinical trials evaluating the value of new anticancer drugs and biological therapy (phase I and phase II studies).

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Rose DM, Hochwald SN, Klimstra DS, et al.: Primary duodenal adenocarcinoma: a ten-year experience with 79 patients. J Am Coll Surg 183 (2): 89-96, 1996. [PUBMED Abstract]
  2. North JH, Pack MS: Malignant tumors of the small intestine: a review of 144 cases. Am Surg 66 (1): 46-51, 2000. [PUBMED Abstract]

Treatment of Small Intestine Leiomyosarcoma

Treatment options:

  1. For resectable primary disease:
    • Radical surgical resection.
  2. For unresectable primary disease:
    • Surgical bypass of obstructing lesion and radiation therapy.
    • Clinical trials evaluating the value of new anticancer drugs and biological therapy.
  3. For unresectable metastatic disease:
    • Palliative surgery.
    • Palliative radiation therapy.
    • Palliative chemotherapy.
    • Clinical trials evaluating the value of new anticancer drugs and biological therapy.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Treatment of Recurrent Small Intestine Cancer

Treatment options:

  1. For metastatic adenocarcinoma or leiomyosarcoma:
    • No standard effective chemotherapy exists for patients with recurrent metastatic adenocarcinoma or leiomyosarcoma of the small intestine. These patients should consider enrolling in phase I or II clinical trials evaluating new anticancer drugs or biological therapy.
  2. For locally recurrent disease:
    • Surgery.
    • Palliative radiation therapy.
    • Palliative chemotherapy.
    • Clinical trials evaluating ways of improving local control, such as the use of radiation therapy with radiosensitizers with or without systemic chemotherapy.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Latest Updates to This Summary (03/06/2025)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

General Information About Small Intestine Cancer

Updated statistics with estimated new cases and deaths for 2025 (cited American Cancer Society as reference 1).

Revised text to state that small intestine cancer accounts for 3.8% of all digestive system malignancies.

This summary is written and maintained by the PDQ Adult Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® Cancer Information for Health Professionals pages.

About This PDQ Summary

Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of small intestine cancer. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.

Reviewers and Updates

This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

Board members review recently published articles each month to determine whether an article should:

  • be discussed at a meeting,
  • be cited with text, or
  • replace or update an existing article that is already cited.

Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.

The lead reviewers for Small Intestine Cancer Treatment are:

  • Amit Chowdhry, MD, PhD (University of Rochester Medical Center)
  • Leon Pappas, MD, PhD (Massachusetts General Hospital)

Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website’s Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

Levels of Evidence

Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.

Permission to Use This Summary

PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”

The preferred citation for this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Small Intestine Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/small-intestine/hp/small-intestine-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389423]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in Visuals Online, a collection of over 2,000 scientific images.

Disclaimer

Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s Email Us.

Small Intestine Cancer—Health Professional Version

Small Intestine Cancer—Health Professional Version

Treatment

PDQ Treatment Information for Health Professionals

More information

Causes & Prevention

NCI does not have PDQ evidence-based information about prevention of small intestine cancer.

Screening

NCI does not have PDQ evidence-based information about screening for small intestine cancer.

Supportive & Palliative Care

We offer evidence-based supportive and palliative care information for health professionals on the assessment and management of cancer-related symptoms and conditions.

Cancer Pain Nausea and Vomiting Nutrition in Cancer Care Transition to End-of-Life Care Last Days of Life View all Supportive and Palliative Care Summaries

Small Intestine Cancer—Patient Version

Small Intestine Cancer—Patient Version

Overview

Small intestine cancer usually begins in an area of the intestine called the duodenum. This cancer is rarer than cancers in other parts of the gastrointestinal system, such as the colon and stomach. Explore the links on this page to learn more about small intestine cancer treatment, statistics, research, and clinical trials.

Treatment

PDQ Treatment Information for Patients

More information

Causes & Prevention

NCI does not have PDQ evidence-based information about prevention of small intestine cancer.

Screening

NCI does not have PDQ evidence-based information about screening for small intestine cancer.

Coping with Cancer

The information in this section is meant to help you cope with the many issues and concerns that occur when you have cancer.

Emotions and Cancer Adjusting to Cancer Support for Caregivers Survivorship Advanced Cancer Managing Cancer Care

Pituitary Tumors Treatment (PDQ®)–Patient Version

Pituitary Tumors Treatment (PDQ®)–Patient Version

General Information About Pituitary Tumors

Key Points

  • A pituitary tumor is a growth of abnormal cells in the tissues of the pituitary gland.
  • The pituitary gland hormones control many other glands in the body.
  • Having certain genetic conditions increases the risk of developing a pituitary tumor.
  • Signs of a pituitary tumor include problems with vision and certain physical changes.
  • Imaging studies and tests that examine the blood and urine are used to diagnose a pituitary tumor.
  • Certain factors affect prognosis (chance of recovery) and treatment options.

A pituitary tumor is a growth of abnormal cells in the tissues of the pituitary gland.

Pituitary tumors form in the pituitary gland, a pea-sized organ in the center of the brain, just above the back of the nose. The pituitary gland is sometimes called the “master endocrine gland” because it makes hormones that affect the way many parts of the body work. It also controls hormones made by many other glands in the body.

EnlargeDrawing of the inside of the brain showing ventricles (fluid-filled spaces), choroid plexus, hypothalamus, pineal gland, pituitary gland, optic nerve, brain stem, cerebellum, cerebrum, medulla, pons, and spinal cord.
Anatomy of the inside of the brain, showing the pineal and pituitary glands, optic nerve, ventricles (with cerebrospinal fluid shown in blue), and other parts of the brain.

Pituitary tumors are divided into three groups:

  • Benign pituitary adenomas: Tumors that are not cancer. These tumors grow very slowly and do not spread from the pituitary gland to other parts of the body.
  • Invasive pituitary adenomas: Benign tumors that may spread to bones of the skull or the sinus cavity below the pituitary gland.
  • Pituitary carcinomas: Tumors that are malignant (cancer). These pituitary tumors spread into other areas of the central nervous system (brain and spinal cord) or outside of the central nervous system. Very few pituitary tumors are malignant.

Pituitary tumors may be either nonfunctioning or functioning.

  • Nonfunctioning pituitary tumors do not make extra amounts of hormones.
  • Functioning pituitary tumors make more than the normal amount of one or more hormones. Most pituitary tumors are functioning tumors. The extra hormones made by pituitary tumors may cause certain signs or symptoms of disease.

The pituitary gland hormones control many other glands in the body.

Hormones made by the pituitary gland include:

Having certain genetic conditions increases the risk of developing a pituitary tumor.

Anything that increases a person’s chance of getting a disease is called a risk factor. Not every person with one or more of these risk factors will develop pituitary tumors, and they will develop in some people who don’t have any known risk factors. Talk with your doctor if you think you may be at risk. Hereditary syndromes that increase a person’s risk for pituitary tumors include:

Signs of a pituitary tumor include problems with vision and certain physical changes.

Signs and symptoms can be caused by the growth of the tumor and/or by hormones the tumor makes or by other conditions. Some tumors may not cause signs or symptoms. Check with your doctor if you have any of these problems.

Signs and symptoms of a nonfunctioning pituitary tumor

Sometimes, a pituitary tumor may press on or damage parts of the pituitary gland, causing it to stop making one or more hormones. Too little of a certain hormone will affect the work of the gland or organ that the hormone controls. The following signs and symptoms may occur:

  • Headache.
  • Some loss of vision.
  • Loss of body hair.
  • In women, less frequent or no menstrual periods or no milk from the breasts.
  • In men, loss of facial hair, growth of breast tissue, and impotence.
  • In women and men, lower sex drive.
  • In children, slowed growth and sexual development.

Most of the tumors that make LH and FSH do not make enough extra hormone to cause signs and symptoms. These tumors are considered to be nonfunctioning tumors.

Signs and symptoms of a functioning pituitary tumor

When a functioning pituitary tumor makes extra hormones, the signs and symptoms will depend on the type of hormone being made.

Too much prolactin may cause:

  • Headache.
  • Some loss of vision.
  • Less frequent or no menstrual periods or menstrual periods with a very light flow.
  • Trouble becoming pregnant or an inability to become pregnant.
  • Impotence in men.
  • Lower sex drive.
  • Flow of breast milk in a woman who is not pregnant or breast-feeding.

Too much ACTH may cause:

  • Headache.
  • Some loss of vision.
  • Weight gain in the face, neck, and trunk of the body, and thin arms and legs.
  • A lump of fat on the back of the neck.
  • Thin skin that may have purple or pink stretch marks on the chest or abdomen.
  • Easy bruising.
  • Growth of fine hair on the face, upper back, or arms.
  • Bones that break easily.
  • Anxiety, irritability, and depression.

Too much growth hormone may cause:

  • Headache.
  • Some loss of vision.
  • In adults, acromegaly (growth of the bones in the face, hands, and feet). In children, the whole body may grow much taller and larger than normal.
  • Tingling or numbness in the hands and fingers.
  • Snoring or pauses in breathing during sleep.
  • Joint pain.
  • Sweating more than usual.
  • Dysmorphophobia (extreme dislike of or concern about one or more parts of the body).

Too much thyroid-stimulating hormone may cause:

  • Irregular heartbeat.
  • Shakiness.
  • Weight loss.
  • Trouble sleeping.
  • Frequent bowel movements.
  • Sweating.

Other general signs and symptoms of pituitary tumors:

Imaging studies and tests that examine the blood and urine are used to diagnose a pituitary tumor.

In addition to asking about your personal and family health history and doing a physical exam, your doctor may perform the following tests and procedures:

  • Eye exam: An exam to check vision and the general health of the eyes.
  • Visual field exam: An exam to check a person’s field of vision (the total area in which objects can be seen). This test measures both central vision (how much a person can see when looking straight ahead) and peripheral vision (how much a person can see in all other directions while staring straight ahead). The eyes are tested one at a time. The eye not being tested is covered.
  • Neurological exam: A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.
  • MRI (magnetic resonance imaging) with gadolinium: A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain and spinal cord. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI).
  • Blood chemistry study: A procedure in which a blood sample is checked to measure the amounts of certain substances, such as glucose (sugar), released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
  • Blood tests: Tests to measure the levels of testosterone or estrogen in the blood. A higher- or lower-than-normal amount of these hormones may be a sign of pituitary tumor.
  • Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of certain substances. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. A higher-than-normal amount of the hormone cortisol may be a sign of a pituitary tumor and Cushing syndrome.
  • High-dose dexamethasone suppression test: A test in which one or more high doses of dexamethasone are given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days. This test is done to check if the adrenal gland is making too much cortisol or if the pituitary gland is telling the adrenal glands to make too much cortisol.
  • Low-dose dexamethasone suppression test: A test in which one or more small doses of dexamethasone are given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days. This test is done to check if the adrenal gland is making too much cortisol.
  • Venous sampling for pituitary tumors: A procedure in which a sample of blood is taken from veins coming from the pituitary gland. The sample is checked to measure the amount of ACTH released into the blood by the gland. Venous sampling may be done if blood tests show there is a tumor making ACTH, but the pituitary gland looks normal in the imaging tests.
  • Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer.

    The following tests may be done on the sample of tissue that is removed:

    • Immunohistochemistry: A laboratory test that uses antibodies to check for certain antigens (markers) in a sample of a patient’s tissue. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to a specific antigen in the tissue sample, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This type of test is used to help diagnose cancer and to help tell one type of cancer from another type of cancer.
    • Immunocytochemistry: A laboratory test that uses antibodies to check for certain antigens (markers) in a sample of a patient’s cells. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to the antigen in the sample of the patient’s cells, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This type of test is used to help diagnose cancer and to help tell one type of cancer from another type of cancer.
    • Light and electron microscopy: A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis depends on the type of tumor and whether the tumor has spread into other areas of the central nervous system (brain and spinal cord) or outside of the central nervous system to other parts of the body.

Treatment options depend on:

  • The type and size of the tumor.
  • Whether the tumor is making hormones.
  • Whether the tumor is causing problems with vision or other signs or symptoms.
  • Whether the tumor has spread into the brain around the pituitary gland or to other parts of the body.
  • Whether the tumor has just been diagnosed or has recurred (come back).

Stages of Pituitary Tumors

Key Points

  • Once a pituitary tumor has been diagnosed, tests are done to find out if it has spread within the central nervous system (brain and spinal cord) or to other parts of the body.
  • Pituitary tumors are described in several ways.
  • Pituitary tumors can recur (come back) after they have been treated.

Once a pituitary tumor has been diagnosed, tests are done to find out if it has spread within the central nervous system (brain and spinal cord) or to other parts of the body.

The extent or spread of cancer is usually described as stages. There is no standard staging system for pituitary tumors. Once a pituitary tumor is found, tests are done to find out if the tumor has spread into the brain or to other parts of the body. The following test may be used:

  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).

Pituitary tumors are described in several ways.

EnlargeDrawing shows different sizes of a tumor in centimeters (cm) compared to the size of a pea (1 cm), a peanut (2 cm), a grape (3 cm), a walnut (4 cm), a lime (5 cm), an egg (6 cm), a peach (7 cm), and a grapefruit (10 cm). Also shown is a 10-cm ruler and a 4-inch ruler.
Tumor sizes are often measured in centimeters (cm) or inches. Common food items that can be used to show tumor size in cm include: a pea (1 cm), a peanut (2 cm), a grape (3 cm), a walnut (4 cm), a lime (5 cm or 2 inches), an egg (6 cm), a peach (7 cm), and a grapefruit (10 cm or 4 inches).

Pituitary tumors are described by their size and grade, whether or not they make extra hormones, and whether the tumor has spread to other parts of the body.

The following sizes are used:

  • Microadenoma: The tumor is smaller than 1 centimeter.
  • Macroadenoma: The tumor is 1 centimeter or larger.

Most pituitary adenomas are microadenomas.

The grade of a pituitary tumor is based on how far it has grown into the surrounding area of the brain, including the sella (the bone at the base of the skull, where the pituitary gland sits).

Pituitary tumors can recur (come back) after they have been treated.

The cancer may come back in the pituitary gland or in other parts of the body.

Treatment Option Overview

Key Points

  • There are different types of treatment for patients with pituitary tumors.
  • The following types of treatment are used:
    • Surgery
    • Radiation therapy
    • Drug therapy
    • Chemotherapy
  • New types of treatment are being tested in clinical trials.
  • Treatment for pituitary tumors may cause side effects.
  • Patients may want to think about taking part in a clinical trial.
  • Patients can enter clinical trials before, during, or after starting their cancer treatment.
  • Follow-up tests may be needed.

There are different types of treatment for patients with pituitary tumors.

Different types of treatments are available for patients with pituitary tumors. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

The following types of treatment are used:

Surgery

Many pituitary tumors can be removed by surgery using one of the following operations:

  • Transsphenoidal surgery: A type of surgery in which the instruments are inserted into part of the brain by going through an incision (cut) made under the upper lip or at the bottom of the nose between the nostrils and then through the sphenoid bone (a butterfly-shaped bone at the base of the skull) to reach the pituitary gland. The pituitary gland lies just above the sphenoid bone.
    EnlargeTranssphenoidal surgery; drawing shows an endoscope and a curette inserted through the nose and sphenoid sinus to remove cancer from the pituitary gland. The sphenoid bone is also shown.
    Transsphenoidal surgery. An endoscope and a curette are inserted through the nose and sphenoid sinus to remove cancer from the pituitary gland.
  • Endoscopic transsphenoidal surgery: A type of surgery in which an endoscope is inserted through an incision (cut) made at the back of the inside of the nose and then through the sphenoid bone to reach the pituitary gland. An endoscope is a thin, tube-like instrument with a light, a lens for viewing, and a tool for removing tumor tissue.
  • Craniotomy: Surgery to remove the tumor through an opening made in the skull.
    EnlargeDrawing of a craniotomy showing a section of the scalp that has been pulled back to remove a piece of the skull; the dura covering the brain has been opened to expose the brain. The layer of muscle under the scalp is also shown.
    Craniotomy: An opening is made in the skull and a piece of the skull is removed to show part of the brain.

After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. This type of radiation therapy may include:

  • Stereotactic radiosurgery: A rigid head frame is attached to the skull to keep the head still during the radiation treatment. A machine aims a single large dose of radiation directly at the tumor. This procedure does not involve surgery. It is also called stereotaxic radiosurgery, radiosurgery, and radiation surgery.

Drug therapy

Drugs may be given to stop a functioning pituitary tumor from making too many hormones.

Chemotherapy

Chemotherapy may be used as palliative treatment for pituitary carcinomas, to relieve symptoms and improve the patient’s quality of life. Chemotherapy uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type of the cancer being treated.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI website.

Treatment for pituitary tumors may cause side effects.

For information about side effects caused by treatment for cancer, visit our Side Effects page.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today’s standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

Follow-up tests may be needed.

As you go through treatment, you will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back).

Treatment of Nonfunctioning Pituitary Tumors

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment may include:

Treatment for luteinizing hormone–producing and follicle-stimulating hormone–producing tumors is usually transsphenoidal surgery to remove the tumor.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Prolactin-Producing Pituitary Tumors

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment may include:

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of ACTH-Producing Pituitary Tumors

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment may include:

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Growth Hormone–Producing Pituitary Tumors

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment may include:

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Thyroid-Stimulating Hormone–Producing Tumors

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment may include:

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Pituitary Carcinomas

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of pituitary carcinomas is palliative, to relieve symptoms and improve the quality of life. Treatment may include:

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Recurrent Pituitary Tumors

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment may include:

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

To Learn More About Pituitary Tumors

About This PDQ Summary

About PDQ

Physician Data Query (PDQ) is the National Cancer Institute’s (NCI’s) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.

PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of pituitary tumors. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

Reviewers and Updates

Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary (“Updated”) is the date of the most recent change.

The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.

Clinical Trial Information

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become “standard.” Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Clinical trials can be found online at NCI’s website. For more information, call the Cancer Information Service (CIS), NCI’s contact center, at 1-800-4-CANCER (1-800-422-6237).

Permission to Use This Summary

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Pituitary Tumors Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/pituitary/patient/pituitary-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389369]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.