Vaginal Cancer: The Basics

Neha Vapiwala, MD and Eric T. Shinohara, MD, MSCI and Carolyn Vachani, RN, MSN, AOCN
Abramson Cancer Center of the University of Pennsylvania
Last Modified: July 21, 2014

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What is vaginal cancer?

Vaginal cancer is an abnormal growth of malignant (cancerous) cells in the vagina. The vagina itself, sometimes referred to as the "birth canal", is a 3 - 4 inch hollow tube that runs from the vulva (outside genitalia) up to the cervix (the lower part of the uterus, or womb). The walls of the vagina are often in a "closed" or collapsed position, but are able to expand significantly during sexual activity or delivery of a baby.

What are the different types of vaginal cancer?

The vast majority of vaginal cancers (~85%) are squamous cell carcinomas, which grow in the "skin" (epithelial lining) of the vagina. They usually occur in the top part of the vagina near the cervix, and evolve over a period of many years from precancerous areas called vaginal intraepithelial neoplasia (VAIN).

A much smaller percentage of vaginal cancers (5-10%) are adenocarcinomas, which arise from glandular tissues. A subtype of these is clear cell adenocarcinoma, which most often occurs in young women whose mothers took an old hormonal medication called diethylstilbestrol (DES) while they were pregnant with them. These women are sometimes referred to as "DES daughters." Diethylstilbestrol was prescribed in the US from the 1940s to early 1970s for prevention of miscarriages.

Much rarer types of vaginal cancer include melanomas (2-3%), seen in the lower or outer portion of the vagina, sarcomas (2-3%), and even rarer types including small cell, lymphoma and carcinoid. These subtypes will not be addressed in this article.

The most common cancers in the vaginal are actually not "primary" tumors (or tumors that start in the vagina), but metastasis (cancers that have spread from another site in the body to the vagina). This can be caused by either direct growth of a tumor into the vagina (for example, from the endometrium, rectum or bladder) or from a distant site (for example, from the breast or ovary) by traveling through the blood stream or lymph system.

How common is vaginal cancer?

It is a rare cancer, representing only about 1% of all gynecologic tumors. There are about 3,170 new cases reported each year in the US with about 880 deaths attributed to the disease.

What are the risk factors for vaginal cancer?

Typically this is a condition affecting older women, with an average age of 60 years old at diagnosis. Almost half of cases are diagnosed in women over 70 years of age. Adenocarcinomas of the vagina, particularly the clear cell variant mentioned above, can be seen in younger women and commonly present before the age of 20 and are associated with DES exposure in the womb (discussed below).

Squamous cell cancer of the vagina is associated with certain high-risk strains of the human papillomavirus (HPV). In fact, having a diagnosis of cervical cancer, which is also caused by high-risk strains of HPV, is an important risk factor for developing vaginal cancer.

HPV

HPV is a sexually transmitted disease that is incredibly common in the population. Most college-aged men and women have been exposed to HPV, though in most, the immune system inactivates or clears the virus from the body. There are over 100 different subtypes, or strains, of HPV and only certain subtypes are "oncogenic," or able to cause cancer. Certain strains of HPV cause genital warts, though these strains are not oncogenic. Infection with HPV typically causes no symptoms, and may only be detected when a woman has an abnormal pap result or HPV testing that may be done along with the pap test. It is important to know that only a very small percentage of women who have a high-risk strain of HPV will develop a cancer caused by the virus; so simply having HPV does not mean that you will get cancer.

Women who have had multiple male sexual partners, began having sexual intercourse at an early age, or have had male sexual partners who are considered high risk (meaning that they have had many sexual partners and/or began having sexual intercourse at an early age) are at a higher risk for developing an HPV-related cancer.

Being a current smoker is also considered a risk factor. Smoking is linked to an inability for the body's immune system to clear an HPV infection, therefore smokers are more likely to develop chronic HPV infections that may lead to a cancer. Chronic vaginal irritation has also been linked to some cases.

Diethylstilbestrol (DES) Exposure

DES was the first synthetic estrogen and was given to pregnant women in the US from 1938-1971 because it was believed to prevent miscarriages and promote "healthy pregnancies." It was found that not only did the drug not prevent problems associated with pregnancy, it also caused health issues for the women taking it, as well as children born of these pregnancies. These include both female ("DES daughters") and male children, though the cancer risks have only been proven in female offspring.

DES daughters, as they have become known, are at risk for developing a rare form of vaginal or cervical cancer, called clear cell adenocarcinoma, at a young age (typically before age 20). Though the risk is 40 times that of someone not exposed to DES, which sounds frightening, this translates to 1 in every 1000 women exposed, or 0.1%, so it is still a rare occurrence. This risk is thought to be life-long, therefore women exposed to DES while in utero should be sure they follow annual screening guidelines for "DES daughters." In addition, DES daughters are at an increased risk for breast cancer and should have annual mammograms beginning no later than age 40, along with an annual breast exam by their healthcare provider. It may be helpful to perform monthly self-breast exams to become familiar with their breast tissue and report any changes to their healthcare provider.

What are the symptoms of vaginal cancer?

The most common symptom is painless vaginal bleeding, unrelated to menstrual periods. Bleeding after intercourse may also be a sign of vaginal cancer. Vaginal bleeding in a postmenopausal woman is concerning and should be promptly evaluated. Other symptoms can include vaginal discharge, and painful sexual intercourse. In more advanced vaginal cancers, there may also be bowel symptoms such as blood in the stool, painful bowel movements or constipation, due to tumor invasion into the rectum. Vaginal cancers can also spread locally to the bladder causing painful or difficult urination.

How is vaginal cancer diagnosed?

One of the most important steps in evaluating a patient with gynecologic complaint is a proper pelvic examination. The healthcare provider (HCP) examines the uterus, ovaries, fallopian tubes, and vagina by feeling the areas with their hands and examining areas that can be seen. The bladder and rectum should also be evaluated for any abnormalities. This may require cystoscopy or proctosigmoidoscopy, which uses a camera to examine the inside of the bladder and rectum/bowel, respectively.

Radiology tests, including CT scan, MRI and PET scans may be done to look for enlarged lymph nodes, kidney/bladder problems, liver, or other areas of potential spread of the cancer (metastasis).

A Pap test should be performed, where the outside of the cervix and vagina are scraped and samples are submitted for microscopic analysis and HPV testing. Even if the suspected diagnosis is vaginal cancer, the Pap smear is especially important to rule out cervical cancer, which is much more common than vaginal cancer. Up to 20% of vaginal cancers are found incidentally during cervical cancer screening with a Pap smear.

During colposcopy, the HCP inserts a device with binocular magnifying lenses into the vagina to better visualize the cervix and the inside of the vagina. Any suspicious areas on the cervix and/or along the vaginal walls should be biopsied and sent for microscopic analysis. Any suspicious areas should be tested by applying a dilute solution of acetic acid to the region; abnormal areas typically turn white, making them easier to identify and biopsy.

Once a diagnosis is confirmed, the vaginal cancer is staged. Staging helps the HCP better understand the prognosis and decide which treatment options would be best for each individual. Unlike many cancer types that are not staged until after surgery, vaginal cancer is staged based on the results of the physical exam, radiology tests and any biopsies. This is called "clinical staging" and it is used because many women with vaginal cancer will not undergo surgery as the first treatment.

There are two staging systems; the American Joint Committee on Cancer stage (TNM model) and the FIGO system (Federation Internationale de Gynecologie et d'Obstetrique).

Most gynecologists prefer the FIGO system, which has 4 stages, from stage I (earliest) to stage 4 (most advanced). They are defined as follows:

  • FIGO Stage I: Cancer has started to grow into the deeper tissues of the vagina but has not spread beyond the vaginal wall.
  • FIGO Stage II: Cancer has started to spread outside the vagina into the surrounding tissues BUT has not reached the walls of the pelvis.
  • FIGO Stage III: Cancer has spread outside the vagina and reached nearby lymph nodes or pelvic side walls.
  • FIGO Stage IV: Advanced vaginal cancer, with spread to other body organs outside the vagina.

For further reference, the detailed TNM Categories are shown below (with the FIGO stages shown where they correspond):

Primary tumor (T)

  • TX: Primary tumor cannot be assessed
  • T0: No evidence of primary tumor
  • Tis: Carcinoma in situ (preinvasive)
  • T1 (FIGO I): Tumor confined to vagina
  • T2 (FIGO II): Tumor invades paravaginal tissues but not to pelvic wall*
  • T3 (FIGO III): Tumor extends to pelvic wall
  • T4 (FIGO IVA): Tumor invades mucosa of the bladder or rectum and/or extends beyond the true pelvis

Regional Lymph Nodes (N)

  • NX: Regional nodes cannot be assessed
  • N0: No regional lymph node metastasis
  • N1 (FIGO III): Pelvic or inguinal lymph node metastasis

Distant metastasis (M)

  • MX: Distant metastasis cannot be assessed
  • M0: No distant metastasis
  • M1 (FIGO IVB): Distant metastasis

When using the AJCC TNM system, the stage is reported by combining these values. For example, T3N1M0 would correspond with a FIGO stage III.

How is vaginal cancer treated?

Surgery, radiation therapy and chemotherapy are the typical treatment options, and can be used as single modality therapies or in combination.

There is no "standard" treatment for vaginal cancer and each woman's treatment plan should be based on her particular case. Treatment decisions should take into account the patient's stage of disease, age, other medical history, and personal preference, among other things.

Surgery can be done to remove either part or all of the vagina (called vaginectomy). Generally, small lesions in the upper vagina are the best candidates for surgery. Surgery may include hysterectomy and removal of the vagina and local lymph nodes. In many cases, radiation therapy is an alternative to surgery. In some cases, chemotherapy may be given prior to surgery (called neoadjuvant chemotherapy) to shrink the tumor before removal.

Women who undergo vaginectomy may be candidates for reconstruction. The surgeon creates a vaginal canal using a skin or muscle flap taken from another area of the body.

Radiation therapy uses high-energy rays to kill cancer cells. It is the treatment of choice for most patients with invasive vaginal cancer, especially in stage II disease and higher. It can be delivered as external beam radiation (from an external machine), brachytherapy (using "seeds" of radioisotopes through thin plastic tubes directly into the cancerous area), or more often a combination of both. Occasionally, brachytherapy alone can be used in small cancers in the upper part of the vagina. Generally, if patients have a recurrence after radiation, surgery is the preferred treatment when possible.

Chemotherapy uses medications to kill cancer cells. Given the relative rarity of this disease, there are no randomized clinical trial data supporting the use of chemotherapy together with radiation for vaginal cancer. However, based on the multiple studies in cervical cancer showing better results with the combination compared to radiation alone, many HCPs recommend use of concurrent radiation and cisplatin-based chemotherapy for high-risk vaginal cancer patients. Chemotherapy can also be used to control (as opposed to cure) recurrent or widespread disease.

Side Effects

Many of the side effects from surgery and radiation occur due to the close proximity of the bladder and rectum to the vagina. Due to this close proximity, these organs can be damaged during surgery or with radiation. Side effects from the radiation can include irritation of the bowel and bladder resulting in diarrhea and increased frequency or urgency of bowel movements or urination. This typically resolves within a few weeks of finishing treatment, though it can become a long-term concern for some women.

Radiation can cause scar tissue to form in the vagina and the tissue can become dry and less elastic. There may be some shrinking of the vagina and vaginal opening. Scarring of the vaginal tissue can result in "adhesions", or areas where scar tissue forms, sealing the sides of the vaginal together. This can make it difficult for the doctor to perform vaginal exams and makes sexual intercourse difficult and uncomfortable. Your oncology team will teach you to use vaginal dilators to reduce the severity of this side effect. Rarely, a connection between the bladder or rectum and the vagina can form (also known as a fistula), which allows passage of stool or urine into the vagina.

Damage to the drainage (lymphatic) system in the area, by radiation or surgery to remove lymph nodes, can lead to a chronic swelling called lymphedema, which can occur at any time after treatment. Notify your healthcare provider if you develop any swelling in the legs or pelvis. A survivor with lymphedema who develops pain or redness in the leg(s), especially with fever, should be evaluated right away, as these signs may indicate infection.

Follow-Up Care

After treatment for vaginal cancer, you will be followed closely by your treating physician. In general, you will have a pelvic exam and pap test every 3-6 months for the first 5 years and less frequently thereafter. You should feel comfortable in contacting your care provider for any concerning symptoms and quality of life issues, including body image and sexuality concerns.

After treatment, talk with your oncology team about receiving a survivorship care plan, which can help you manage the transition to survivorship and learn about long-term concerns and life after cancer. You can create your own survivorship care plan on OncoLink.

References and Further Resources

DES Action: advocacy organization for people affected by DES exposure with lots of educational information on their website.

Eyes On The Prize: information and emotional support for those affected by gynecologic cancers. Has a helpful discussion board where you can "chat" with other women.

Society of Gynecologic Oncology: Professional organization of gynecologic oncologists. Find a specialist tool.

Bardawil, T (Ed.). Vaginal Cancer, Medscape, Updated May 3, 2012.

Berek JS, Hacker NF (Eds). Practical Gynecologic Oncology, 3rd ed, Lippincott Williams & Wilkins, Philadelphia, 2000.

FIGO Committee on Gynecologic Oncology: Current FIGO staging for cancer of the vagina, fallopian tube, ovary, and gestational trophoblastic neoplasia. Int J Gynaecol Obstet 105 (1): 3-4, 2009.

Nasu, K et al. (2010) Primary mucinous adenocarcinoma of the vagina. European Journal of Gynaecologic Oncology: 31(6): 679-681.

UpToDate: Vaginal Cancer, Version 14.0

Vagina. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 387-9.


News
Long-Term Cervical/Vaginal CA, Death Risk Up With Treated CIN3

Jan 17, 2014 - For women previously treated for cervical intraepithelial neoplasia grade 3, the risk of acquiring or dying from invasive cervical or vaginal cancer is elevated, particularly among older women, according to a study published online Jan. 14 in BMJ.



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