All About Vaginal Cancer

Author: OncoLink Team
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What is the vagina?

The vagina also called 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 widen during sex or delivery of a baby.

What is vaginal cancer?

Vaginal cancer is an abnormal growth of malignant (cancerous) cells in the vagina. Most vaginal cancers, about 85%, are squamous cell carcinomas. These grow in the "skin" (epithelial lining) of the vagina. They are often found in the top part of the vagina near the cervix. They grow over many years from precancerous areas called vaginal intraepithelial neoplasia (VAIN).

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

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

Most vaginal cancers 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 bloodstream or lymph system.

What causes vaginal cancer and am I at risk?

Vaginal cancer is rare and makes up only about 1% of all gynecologic tumors. The American Cancer Society estimates there will be around 6,230 women diagnosed with vaginal cancer each year in the United States.

Vaginal cancer tends to occur more often in older women.  The average age at diagnosis is 60 years old. Almost half of the 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. Clear cell adenocarcinomas of the vagina are also associated with DES exposure in the womb (discussed below) and can present as early as age 20 but also well into the ’40s.

HPV 

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 is a sexually transmitted infection that is very common. 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. Only certain subtypes are "oncogenic," or able to cause cancer. Infection with HPV often 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. Very few women who have a high-risk strain of HPV will develop a cancer caused by the virus. 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.

Smoking

Smoking is also 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. 

Diethylstilbestrol (DES) Exposure

DES was the first synthetic estrogen. It 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 occurred in female offspring.

DES daughters, as they have become known, are at risk for developing clear cell adenocarcinoma, at a young age (typically before age 20-though cancer can occur well until the woman’s ’40s). 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. As a result, 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.

Other factors that may increase the risk for vaginal cancer include a previous diagnosis of cervical cancer or cervical dysplasia and HIV positive status.

How can I prevent vaginal cancer?

You may be able to lower your risk of vaginal cancer by avoiding HPV exposure, and by stopping or never starting to smoke. A Pap smear may pick up early signs of vaginal cancer, so having this exam performed regularly is suggested (every 3-5 years depending on age and HPV status).

What are the signs 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 woman with a gynecologic complaint is a pelvic examination. During this exam, a healthcare provider (HCP) examines the uterus, ovaries, fallopian tubes, and vagina by feeling the areas with their hands and looking at 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 used 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 done. During a Pap test, the outside of the cervix and vagina are scraped with a tool. The samples are looked at under a microscope and tested for HPV. Even if your provider thinks you have vaginal cancer, the Pap smear is important to rule out cervical cancer. Up to 20% of vaginal cancers are found during cervical cancer screening with a Pap smear.

A colposcopy may be done. During colposcopy, the provider inserts a device with binocular magnifying lenses into the vagina to look at 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.

How is vaginal cancer staged?

Once a diagnosis is confirmed, the vaginal cancer is staged. Staging helps the provider 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.

In order to guide treatment and offer some insight into prognosis, vaginal cancer is staged into four different groups. The staging system used for vaginal cancer is the FIGO system (International Federation of Gynecologists and Obstetricians). Healthcare providers also use the TNM system (also called tumor - node - metastasis system). This system describes the size and locally invasiveness of the tumor (T), which, if any, lymph nodes are involved (N), and if it has spread to other more distant areas of the body (M). This is then interpreted as a stage somewhere from I (one) denoting more limited disease to IV (four) denoting more advanced disease. The TNM breakdown is quite technical and is provided in the appendix for your review.

How is vaginal cancer treated?

Surgery, radiation therapy, and chemotherapy are the typical treatment options. These 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 the 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). Often, patients with 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. In some cases, 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 is 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 the use of concurrent radiation and cisplatin-based chemotherapy for high-risk vaginal cancer patients. Other chemotherapy medications that are used in the treatment of vaginal cancer include carboplatinfluorouracilpaclitaxel, and docetaxel. Chemotherapy can also be used to control (as opposed to cure) recurrent or widespread disease.

Side Effects

Many of the side effects of surgery and radiation happen because the bladder and rectum are close to the vagina. These organs can be damaged during surgery or with radiation. Side effects from the radiation can include irritation of the bowel and bladder which can lead to diarrhea and increased frequency or urgency of bowel movements or urination. This often 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 vaginal exams and 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.

Clinical Trials

Clinical trials are extremely important in furthering our knowledge of this disease. It is through clinical trials that we know what we do today, and many exciting new therapies are currently being tested. Talk to your healthcare provider about participating in clinical trials in your area. You can also explore currently open clinical trials using the OncoLink Clinical Trials Matching Service.

Follow-up Care and Survivorship

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

Fear of recurrence, relationships, and sexual health issues, the financial impact of cancer treatment, employment issues, and coping strategies are common emotional and practical issues experienced by vaginal cancer survivors. Your healthcare team can identify resources for support and management of these challenges faced during and after cancer.

Cancer survivorship is a relatively new focus of oncology care. With some 17 million cancer survivors in the US alone, there is a need to help patients transition from active treatment to survivorship. What happens next, how do you get back to normal, what should you know and do to live healthy going forward? A survivorship care plan can be a first step in educating yourself about navigating life after cancer and helping you communicate knowledgeably with your healthcare providers. Create a survivorship care plan today on OncoLink.

Resources for More Information

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

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

Appendix: Complete Vaginal Cancer Staging

American Joint Committee on Cancer (8th Ed.) & FIGO Staging System

Primary Tumor (T)

FIGO Stage

Description

Tx

 

Primary tumor cannot be assessed

T0

 

No evidence of primary tumor

T1

I

Tumor confined to the vagina

T1a

I

Tumor confined to the vagina, measuring ≤2.0 cm

T1b

I

Tumor confined to the vagina, measuring ≥2.0 cm

T2

II

Tumor invading paravaginal tissues but not to pelvic sidewall

T2a

II

Tumor invading paravaginal tissues but not to pelvic sidewall, measuring ≤2.0 cm

 

 

Tumor invading paravaginal tissues but not to pelvic sidewall. Measuring ≥2.0 cm

T3

III

Tumor extending to pelvic sidewall* and/or causing hydronephrosis or nonfunctioning kidney

T4

IVA

 

*Pelvic sidewall is defined as the muscle, fascia, neurovascular structures, or skeletal portions of the bony pelvis. On rectal examination there is no cancer-free space between the tumor and pelvic side wall.

Regional Lymph Nodes (N)

FIGO Stage

Description

NX

 

Regional lymph nodes cannot be assessed

N0

 

No regional lymph node mestastsis

N0(i+)

 

Isolated tumor cells in regional lymph nodes no greater than 0.2mm

N1

III

Pelvic or inguinal lymph node metastasis

 

Distant Metastasis (M)

FIGO Stage

Description

cM0

 

No distant metastasis

cM1

IVB

Distant metastasis

CM2

IVB

Distant metastasis, microscopically confirmed.

 

Stage Grouping

T

N

M

IA

T1a

N0

M0

IB

T1b

N0

M0

IIA

T2a

N0

M0

IIB

T2b

N0

M0

III

T1-T3

N1

M0

III

T3

N0

M0

IVA

T4

Any N

M0

IVB

Any T

Any N

M1

References

American Cancer Society, Vaginal Cancer

http://www.cancer.org/cancer/vaginalcancer/index

United States Preventive Services Task Force Cervical Cancer Screening Guidelines

http://www.uspreventiveservicestaskforce.org/Page/Document/UpdateSummaryFinal/cervical-cancer-screening

AJCC (2018). Cancer Staging Form Supplement to the AJCC Cancer Staging Manual, 8thEdition. Retrieved from https://cancerstaging.org/references-tools/deskreferences/Documents/AJCC%20Cancer%20Staging%20Form%20Supplement.pdf

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

Beriwal, S., Demanes, D. J., Erickson, B., Jones, E., Jennifer, F., Cormack, R. A., ... & Viswanathan, A. N. (2012). American Brachytherapy Society consensus guidelines for interstitial brachytherapy for vaginal cancer. Brachytherapy11(1), 68-75.

de Martel, C., Plummer, M., Vignat, J., & Franceschi, S. (2017). Worldwide burden of cancer attributable to HPV by site, country and HPV type. International journal of cancer141(4), 664-670.

Hacker, N. F., Eifel, P. J., & van der Velden, J. (2015). Cancer of the vagina. International Journal of gynecology & obstetrics131, S84-S87.

Nasu, K et al. (2010) Primary mucinous adenocarcinoma of the vagina. European journal of gynecologic oncology: 31(6): 679-681.

Ozgul, N., Basaran, D., Boyraz, G., Salman, C., & Yuce, K. (2016). Radical Hysterectomy and Total Abdominal Vaginectomy for Primary Vaginal Cancer. International journal of gynecological cancer26(3), 580-581.

Pannu, H. K. (2014). Vaginal Cancer. In, Atlas of gynecologic oncology imaging (pp. 105-132). Springer New York.

Sinno, A. K., Saraiya, M., Thompson, T. D., Hernandez, B. Y., Goodman, M. T., Steinau, M., ... & Wilkinson, E. J. (2014). Human papillomavirus genotype prevalence in invasive vaginal cancer from a registry-based population. Obstetrics and gynecology123(4), 817.

 

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