The vagina is a 3 to 4 inch (7 ½ -10 cm) tube that at one end joins the cervix, the lower part of the uterus (womb), and at the other end opens onto the vulva, the external genitalia. The vagina is sometimes called the birth canal. The walls of the vagina are lined by a thin layer called the epithelium. The epithelium is formed by squamous epithelial cells. The part of the vaginal wall underneath the epithelium contains connective tissue, involuntary muscle tissue, and lymph vessels and nerves. The vagina is usually in a collapsed state with its walls touching each other. The vaginal walls have many folds that help the vagina to open and expand during sexual intercourse or birth of a baby. Glands in the cervix secrete mucus to keep the vaginal lining moist.
There are several types of vaginal cancer: About 85%-90% of vaginal cancers are squamous cell carcinomas that begin in the epithelial lining of the vagina. They tend to occur in the upper area of the vagina near the cervix. Verrucous carcinoma is a rare type of squamous cell carcinoma that tends to grow slowly. It grows mostly toward the inside of the vagina, and often appears as warty or cauliflower-like lumps. Compared with other squamous cell carcinomas, it is much less likely to invade deeply through the vaginal wall or spread (metastasize) to other organs and has a relatively good prognosis (outlook for cure).
Vaginal squamous cell carcinomas do not appear suddenly, they develop over a period of many years from precancerous changes called vaginal intraepithelial neoplasia (often abbreviated as VAIN).
About 5% to 10% of vaginal cancers are adenocarcinomas. The usual type of vaginal adenocarcinoma typically develops in women older than 50. One special type, called clear cell adenocarcinoma, occurs more often in young women who were exposed to diethylstilbestrol (DES) in utero- (when they were in their mother's womb).
In the past some pregnant women were given DES to prevent miscarriage. The drug became available during the late 1940s and was banned in the USA in 1971. See the section on risk factors for more information on DES and clear cell carcinoma.
Malignant melanoma is a cancer that develops from pigment-producing cells called melanocytes. These cancers usually are found on sun-exposed areas of the skin but occasionally form on the vagina or other internal organs. They account for about 2% to 3% of all vaginal cancers. Melanoma tends to affect the lower or outer portion of the vagina. The tumors show considerable variation in size, color and growth pattern.
About 2% to 3% of vaginal cancers are sarcomas. These cancers form deep in the wall of the vagina, not on its surface epithelium. There are several types of vaginal sarcomas. The most common, leiomyosarcoma, typically affects women older than 50. Leiomyosarcomas resemble the involuntary muscle cells of the vaginal wall. Rhabdomyosarcoma is a childhood cancer, usually found before the age of 3. Its cells resemble voluntary muscle cells - a tissue not normally found in the vaginal wall.
Cancers found in the vagina are actually less common than cancers which start in other organs (such as the uterus, rectum, or bladder) and secondarily spread to the vagina. This document refers only to primary vaginal cancers (those starting in the vagina).
WHAT ARE THE KEY STATISTICS ABOUT CANCER OF THE VAGINA?
Vaginal cancer is rare and is responsible for only about 2% of cancers of the female reproductive system.
The American Cancer Society estimates that in 1998 about 2,000 new cases of vaginal cancer will be diagnosed in the United States.
An estimated 600 women, will die of vaginal cancer in 1998.
DO WE KNOW WHAT CAUSES VAGINAL CANCER?
The exact cause of most vaginal cancers is not known. However, scientists have found that the disease is associated with a number of other conditions, which are described in the section on risk factors. A great deal of research is now underway to learn more about how these risk factors cause cells of the vagina to become cancerous.
Research has shown that substances called tumor suppressor gene products are produced by normal cells to prevent them from growing too rapidly and becoming cancers. Two proteins (E6 and E7) produced by high-risk HPV types can interfere with the functioning of known tumor suppressor gene products.
As mentioned in the section on risk factors, women exposed to diethylstilbestrol (DES) as a fetus (that is, their mothers took DES during pregnancy) are at increased risk for developing clear cell carcinoma. DES clearly increases the likelihood of vaginal adenosis (gland-type cells in the vaginal lining rather than the usual squamous cells). Studies suggest that although the vast majority of women with vaginal adenosis never develop vaginal clear cell carcinoma, those with an uncommon type of adenosis (atypical tuboendometrial adenosis) have an increased risk of developing this cancer.
HOW IS VAGINAL CANCER TREATED?
After the diagnostic tests are done, your cancer care team will recommend a treatment plan. Don't feel rushed about considering your options. If there is anything you do not understand, ask to have it explained again. The choice of treatment depends largely on the type of cancer and stage of the disease when it is diagnosed. Other factors might play a part in choosing the best treatment plan. These might include your age, your overall state of health, whether you plan to have more children, and other personal considerations. Be sure you understand all the risks and side effects of the various therapies before making a decision about treatment.
You may want to get a second opinion. This can provide more information and help you feel confident about the treatment plan you choose. Some insurance companies require a second opinion before they will pay for treatments.
The two main methods of treatment of vaginal cancer are with radiation therapy and surgery. Sometimes, both of these treatments are used together. Chemotherapy is rarely used in treating vaginal cancer.
Whenever possible, treatment is given with the intention of completely removing or destroying the cancer. If a cure is not possible, the goal may be to remove or destroy much of the cancer in order to prevent the tumor from growing, spreading, or returning for as long as possible. If the cancer has spread widely, the main goal of treatment is palliation (relieving pain, blockage of the urinary or intestinal system, or other symptoms).
Radiation therapy uses high-energy rays (or particles) to destroy cancer cells. Radiation therapy is the preferred method of treating most cancers of the vagina.
There are several ways to deliver radiation therapy. The most common way is to carefully focus a radiation beam from a machine outside the body. This is known as external beam radiation or as teletherapy. External beam radiation therapy usually involves having treatments five days a week for a period of six weeks or so. The main drawback of this method is that the radiation can destroy nearby healthy tissue along with the cancerous cells. Some people experience a skin reaction that is like a sunburn on the outside of their skin. This slowly fades away. Other possible side effects include fatigue, nausea, or diarrhea. To reduce the risk of side effects, doctors carefully figure out the exact dose you need and aim the beam as accurately as they can to hit the cancer. In addition to these side effects, higher doses of radiation used in treating more advanced cancers may also cause severe irritation of the intestines and rectum, urethral stricture (narrowing of the tube that carries urine out of the bladder), perforations (holes or tears) of the intestines, and fistulas (abnormal connections) between the vagina and the bladder, rectum, or uterus.
Another method of delivering radiation is to place radioactive material inside the vagina. This may be done in several ways. For low dose brachytherapy (internal radiation therapy) the radioactive material can be placed inside a cylindrical container that is placed in the vagina for a day or two. This method of brachytherapy is called intracavitary radiation. Although gauze packing helps hold the cylinder in place, you have to remain in bed for the course of the treatment. A second type of brachytherapy called interstitial radiation, uses radioactive material inside needles that are placed directly into the cancer.
High dose-rate brachytherapy is given in an outpatient setting. Three or four treatments are given one or two weeks apart. The treatments are often combined with external beam radiation therapy.
The side effects of intracavitary or interstitial radiation therapy depend on the dose of radiation and whether external beam radiation is also given. The radiation may cause vaginal stenosis (shrinkage and narrowing of the vagina), damage to the normal tissue of the vagina, irritation of the intestines and diarrhea.
Surgical procedures are usually reserved for small stage I lesions, for radiation therapy failures, for stage I clear-cell adenocarcinomas and for nonepithelial tumors (sarcomas). The extent of the surgery depends on the size and stage of the cancer. It can range from laser surgery or local excision needed to remove a precancer (VAIN) to radical vaginectomy (removal of the vagina and adjacent tissues), radical hysterectomy (removal of the uterus with adjacent connective tissue and possibly the fallopian tubes and ovaries) and lymphadenectomy (removal of lymph nodes from the groin area or from inside the pelvis near the vagina). If all or most of the vagina must be removed it is possible to reconstruct a vagina with tissue from another part of the body, which will allow a woman to have intercourse. A new vagina can be surgically created out of skin, intestinal tissue, or by myocutaneous (muscle and skin) grafts.
Vaginal cancers are usually treated by radiation therapy. If a woman has already had radiation for cervical cancer, additional radiation might cause severe complications. In this situation it may be necessary to perform pelvic exenteration, a very extensive operation that combines a radical hysterectomy, with removal of the vagina and possibly the bladder, rectum, and part of the colon. The extent of the surgery depends on how far the cancer has spread.
If the bladder is removed, it will be necessary to create a way to store and eliminate urine. This usually involves using a short segment of intestine to function as a new bladder. This may be connected to the abdominal wall so that urine is drained periodically when the woman places a catheter into a urostomy (small opening). Or, urine may drain continuously into a small plastic bag attached to the front of the abdomen. If the rectum and part of the colon is removed, a new way to eliminate solid waste is needed. This is done by attaching the remaining intestine to the abdominal wall so that fecal material can pass through a colostomy (a small opening) into a small plastic bag worn on the front of the abdomen. It may be possible to remove the involved colon and reconnect the free ends of it, so no bags or external appliances are needed.
Chemotherapy is the use of drugs for treating cancer. The drugs can be applied directly to the walls of the vagina (topical chemotherapy), swallowed in pill form, or they can be injected into a vein or muscle. Except for topical creams, chemotherapy is systemic therapy. This means that the drug enters the bloodstream and circulates throughout the body to reach and destroy the cancer cells. So far, systemic chemotherapy has not been very successful in treating vaginal cancer. Its use is generally limited to clinical trials for patients with distant metastases.
Chemotherapeutic drugs given by mouth or injection can reach cancer cells in just about any place inside the body, but can also affect some of the normal, healthy cells in your body. This is what causes the side effects of chemotherapy. Among the possible side effects are:
- Nausea and vomiting
- A decrease or increase in appetite
- Temporary loss of hair
- Mouth sores
- Increased risk of infections
- Increased risk of bleeding from small cuts or scrapes
- Tiredness and reduced capacity for physical work or exercise
Fortunately, most side effects will disappear when your course of chemotherapy ends, but if you have side effects, your cancer care team can suggest steps to ease their impact. For example, there are drugs available to help control nausea and vomiting.
To learn whether a new type of treatment is effective, scientists conduct studies following strict rules. The main questions they want to answer are:
- Is the treatment most likely to be helpful?
- Does this new type of treatment work?
- Does it work better than other treatments already available?
- What side effects does the treatment cause?
- Do the benefits outweigh the risks, including side effects?
- In which patients is the treatment most likely to be helpful?
Studies of promising new or experimental treatments in patients are known as clinical trials. During a course of treatment for vaginal cancer, the doctor may suggest that you take part in a clinical trial of an investigational treatment. A clinical trial is only done when there is some reason to believe that the treatment being studied may be of value to the patient. Treatments used in clinical trials are often found to provide real benefits.
However, there are some risks. No one involved in the study knows in advance whether the treatment will work or exactly what side effects will occur. That is what the study is designed to discover. While most side effects will disappear in time, some can be permanent or even life-threatening. Keep in mind, though, that even standard treatments have side effects and that the disease you have can reduce the length or quality of your life. Depending on many factors, you may decide that a clinical trial will be of value in your case.
Enrollment in any clinical trial is completely up to you. Your doctors and nurses will explain the study to you in detail and will give you a form to read and sign indicating your desire to take part. This process is known as giving you informed consent. Even after signing the form and after the trial begins, you are free to leave the study any time you want, for any reason. Taking part in the study does not prevent you from getting other medical care you may need.
To find out more about clinical trials, ask your cancer care team. Among the questions you should ask are:
- What is the purpose of the study?
- What kinds of tests and treatments does the study involve?
- What does this treatment do?
- What is likely to happen in my case with, or without, this new research treatment?
- What are my other choices and their advantages and disadvantages?
- How could the study affect my daily life?
- What side effects can I expect from the study? Can the side effects be controlled?
- How long will the study last?
- Will I have to be hospitalized? If so, how often and for how long?
- Will the study cost me anything? Will any of the treatment be free?
- If I am harmed as a result of the research, what treatment would I be entitled to?
- What type of long-term follow-up care is part of the study?
You can get a list of current clinical trials by calling the National Cancer Institute's Cancer Information Service toll free at 1-800-4-CANCER.
Treatment Options by Stage and Type of Vaginal Cancer
The type of treatment your cancer care team will recommend depends on the type of vaginal cancer you have and how far the cancer has spread. This section summarizes the choices available according to the stage of your cancer. This information is specific for squamous cell vaginal cancer and adenocarcinoma of the vagina.
Stage 0 squamous cell cancer: The usual treatment options are laser vaporization, loop electroexcision or local excision of the affected areas. If several areas of the vagina are affected, partial or even total vaginectomy may rarely be needed. Intracavitary radiation is sometimes used but shrinkage of the vagina is a possible side effect.
Stage I squamous cell cancer: Radiation therapy is used for most stage I vaginal cancers. If the cancer is less than 5 mm thick (about 3/16 inch) intracavitary radiation is used. If it is thicker than 5 mm, wider than 2 cm (about ¾ inch) and is localized to one wall, both intracavitary and interstitial radiation are used. In some cases external beam radiation may be added as well. Lesions on the upper vagina may be occasionally treated by a radical hysterectomy, bilateral pelvic lymph node removal and radical partial vaginectomy.
Removal of the entire vagina is an option for some cancers (radical vaginectomy) of the lower vagina. Reconstructive surgery to create a new vagina after treatment of the cancer is an option. Postoperative radiation (external beam) may be needed to irradiate microscopic disease in the pelvic nodes or a radical bilateral lymph node dissection may be performed instead.
Stage II squamous cell cancer: The usual treatment is a combination of intracavitary, interstitial and external beam radiation.
Radical surgery (radical vaginectomy or pelvic exenteration) is an option for some patients with stage II vaginal squamous cell cancer. It is used mostly in treating women who have already had radiation therapy for cervical cancer, and who would not be able to tolerate additional radiation without severe damage to normal tissues. Stage III or IVA squamous cell cancer: The usual treatment is radiation therapy which is similar to that given for stage II squamous cell vaginal cancer. Curative surgery is generally not attempted. Rarely, to reduce the risk of side effects, surgery is performed to move parts of the intestines away from the area of the vagina before radiation therapy begins.
Stage IVB squamous cell cancer: Radiation therapy is the usual treatment, but is given with the intent of palliation (relief of symptoms) and is not expected to be curative. Chemotherapy may also be given, usually in the context of a clinical trial.
Stage I adenocarcinoma: The usual surgical treatment is radical hysterectomy, partial or radical vaginectomy, and removal of groin and/or pelvic lymph nodes, followed by reconstructive surgery as desired. Another approach is combined interstitial and intracavitary radiation therapy, with external beam radiation of groin and/or pelvic lymph nodes if the lower part of the vagina is involved.
A newer approach in women wishing to preserve fertility is to combine wide local excision of the cancer, removal of some pelvic and/or groin nodes, and interstitial radiation therapy. This approach is as effective as radical surgery or higher doses of radiation, but has the advantage of preserving fertility in most cases. This is an important consideration, since many women with vaginal adenocarcinoma are young.
Stage II, III and IVA adenocarcinoma: The usual treatment is radiation therapy which is similar to that given for stage II, III, or IVA squamous cell vaginal cancer. Curative surgery is generally not attempted. Sometimes to reduce the risk of side effects, surgery is rarely performed to move parts of the intestines away from the area of the vagina before radiation therapy begins.
Stage IVB adenocarcinoma: Radiation therapy is the usual treatment, but is given with the intent of palliation (relief of symptoms) and is not expected to be curative. Chemotherapy may also be given, usually in the context of a clinical trial.
Recurrent squamous cell cancer or adenocarcinoma of the vagina: If a stage I or stage II vaginal cancer recurs locally (near where the original tumor was) treatment with radical surgery (such as pelvic exenteration) or with radiation therapy (if treated primarily by surgery) may be successful. If the cancer was initially treated with radiation therapy, the recurrence will probably be treated surgically if without evidence of nodal or distant metastases (spread). Surgery is the usual choice when the cancer has come back after radiation therapy. Recurrences of higher stage cancers or distant recurrences of low stage cancers are not, usually curable by currently available treatments. Care focuses mostly on relieving symptoms, although participation in a clinical trial of new treatments may be beneficial.
Melanoma: Because melanoma of the vagina is extremely rare, doctors have been unable to compare enough patients with different treatments to determine the best approach. This cancer does not respond well to chemotherapy or radiation therapy, so surgery is the main treatment. However, doctors are not yet certain about how much surgery is needed. That is, whether removing the cancer and a margin of normal tissue is any less effective than removing the entire vagina with some tissue from nearby organs, and the extent of lymph node removal is debated. Most practitioners would just sample lymph nodes for prognosis.
Patients with metastatic vaginal melanoma may benefit from participation in clinical trials of new treatments such as biologic or gene therapies.
Rhabdomyosarcoma: Treatment of rhabdomyosarcoma is discussed in a separate ACS document.
Prognosis Following Treatment of Vaginal Cancer
|Type and stage of cancer
||5-year survival rate *
|Squamous cell cancer
||70% - 90%
||43% - 60%
||30% - 40%
||15% - 18%
||5% - 20%
|* The range of 5-year survival rates reflects results reported from several studies, which may vary in types of treatment used. Estimated survival rates for adenocarcinoma are based on experience with only a small number of patients.
WHAT SHOULD YOU ASK YOUR DOCTOR ABOUT VAGINAL CANCER?
As you deal with your cancer and the treatment process, you need to have frank, open discussions with your cancer care team. You should feel free to ask any question that's on your mind, no matter how trivial it might seem. Among the questions you might want to ask are:
- What kind of vaginal cancer do I have?
- Has my cancer spread beyond the primary site?
- What is the stage of my cancer? What does the staging mean in my case?
- What treatment choices do I have?
- Based on what you've learned about my cancer, how long do you think I'll survive?
- What side effects can I expect from my treatment?
- How long will it take me to recover from treatment?
- When can I go back to work after treatment?
- Will I be able to have sex after treatment? What reconstructive surgery, if any, will I need?
- What are the chances that my cancer will recur?
- What should I do to be ready for treatment?
- Should I get a second opinion?
You will no doubt have other questions about your own personal situation. Be sure and write your questions down so you remember to ask them during each visit with your cancer care team. Keep in mind, too, that doctors are not the only ones who can provide you with information. Other health care professionals, such as nurses and social workers, may have the answers you seek.