Vaginal Cancer: The Basics

Neha Vapiwala, MD and Eric T. Shinohara, MD, MSCI
Abramson Cancer Center of the University of Pennsylvania
Last Modified: February 23, 2008

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What is vaginal cancer?

It is an abnormal growth of malignant cells (neoplasm, tumor) 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 baby delivery.

What are the different types of vaginal cancer?

The vast majority of vaginal cancers (over 90%) 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%) are adenocarcinomas which arise from glandular tissues. A subtype of these is clear cell adenocarcinoma, which occurs in young women whose mothers took an old hormonal medication called diethylstilbestrol (DES) while they were pregnant with them (these women are referred to as DES Daughters). Diethylstilbestrol was prescribed from the 1940s to early 1970s for prevention of miscarriages.

Much rarer types of vaginal cancer are melanomas (2-3%), seen in the lower or outer portion of the vagina, and sarcomas (2-3%). The most common cause of masses in the vaginal are metastasis (spread of cancer from another site to the vagina). This can be caused by either direct growth of the tumor into the vagina (for example, from the rectum of bladder) or from a distant site (for example, breast) through the blood stream or lymph nodes.

How common is vaginal cancer?

It is a rare cancer, representing only about 2% of all gynecologic tumors. There are about 2,000 new cases reported each year in the US with about 800 deaths attributed to the disease.

Who gets vaginal cancer?

Typically this is a condition affecting older women, with a median age of 65 – 70 years old at diagnosis. The greatest number 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.

What are the risk factors for vaginal cancer?

Squamous cell cancer of the vagina is associated with increasing age and certain high-risk strains of the human papillomavirus (HPV). In fact, having a diagnosis of cervical cancer is itself a big risk factor for developing vaginal cancer. In addition to this, several of the risk factors for cervical cancer have been linked to vaginal cancer as well. These risk factors include: smoking, young age at first intercourse, and a large number of lifetime sexual partners. Chronic vaginal irritation has also been linked to some cases.

As mentioned earlier, clear cell adenocarcinoma of the vagina is associated with DES exposure in the womb.

What are the symptoms of vaginal cancer?

Painless vaginal bleeding, unrelated to menstrual periods, is the most common symptom. Bleeding after intercourse may also be a sign of vaginal cancer. Vaginal bleeding in a postmenopausal woman is cancer until proven otherwise 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) should examine the uterus, ovaries, fallopian tubes, and vagina. Vaginal cancer is diagnosed and staged clinically, and so the bladder and rectum should also be evaluated (with cystoscopy and proctoscopy, if necessary) for any abnormalities. CT and MRI scans of the upper abdomen and pelvis are not currently standard recommendations, but are often done to look for enlarged lymph nodes, kidney/bladder problems, and liver 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.

Colposcopy is where 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 it is diagnosed, how is vaginal cancer staged?

Staging helps doctors decide which treatment options would be best for each individual as well as the prognosis.

Both the American Joint Committee on Cancer stage (TNM model) and the Federation Internationale de Gynecologie et d’Obstetrique (FIGO) can be used.

Most gynecologists prefer the FIGO system, which has 5 stages, from stage 0 (earliest) to stage 4 (most advanced). They are defined as follows:

  • Stage 0 - very earliest stage of vaginal cancer, also known as carcinoma in-situ (CIS), vaginal intraepithelial neoplasia (VAIN), or pre-cancer, because the cancer cells are trapped in the vaginal skin and have not yet grown into the deeper tissues or spread away from the vagina
  • Stage 1 - cancer has started to grow into the deeper tissues of the vagina but has not spread beyond the vagina
  • Stage 2 - cancer has started to spread outside the vagina into the surrounding tissues BUT has not reached the walls of the pelvis
  • Stage 3 - cancer has spread outside the vagina and reached nearby lymph nodes or pelvic side walls
  • Stage 4 - advanced vaginal cancer, with spread to other body organs outside the vagina

For further reference, the detailed TNM Categories/ FIGO Stages are shown below:

Primary tumor (T)

  • TX: Primary tumor cannot be assessed
  • T0: No evidence of primary tumor
  • Tis/ 0: Carcinoma in situ
  • T1/ I: Tumor confined to vagina
  • T2/ II: Tumor invades paravaginal tissues but not to pelvic wall*
  • T3/ III: Tumor extends to pelvic wall
  • T4/ 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/ IVB: Pelvic or inguinal lymph node metastasis

Distant metastasis (M)

  • MX: Distant metastasis cannot be assessed
  • M0: No distant metastasis
  • M1/ IVB: Distant metastasis

Adapted from Vagina. In: American Joint Committee on Cancer: AJCC Cancer Staging Manual. 6th ed. New York, NY: Springer, 2002, pp 251-257.

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.

The optimal treatment regimen should ultimately be individualized as much as possible. It should take into account the patient’s stage of disease, other medical history, and personal preference, among other things.

Surgery can be done to remove either part or all of the vagina. Generally, small lesions in the upper vagina are the best candidates for surgery. Surgical methods include:

  • laser surgery for very early stage disease, using a narrow beam of light to kill cancer cells
  • wide local excision to excise the cancer and some surrounding tissue
  • vaginectomy, where the surgeon removes the vagina and usually some pelvic lymph nodes
  • radical hysterectomy if cancer has spread outside of the vagina, with removal of the uterus, ovaries and fallopian tubes, as well as lymph nodes
  • pelvic exenteration for extremely advanced disease, especially if an abnormal connection (fistula) has formed between the vagina and the bladder or rectum

Radiation therapy uses high-energy rays to kill cancer cells. It is the treatment of choice for most patients with invasive vaginal cancer, especially stage 2 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 if the preferred treatment.

Chemotherapy uses drugs to kill cancer cells. Given the relative rarity of this disease, there are no randomized 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, but results have typically been poor.

Most 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 with increased frequency of bowel movements or urination. Radiation can cause scar tissue to form in the vagina which can make intercourse painful. A dilator to maintain the patency of the vagina is often used to prevent this. 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.

What is the prognosis?

Squamous cell and adenocarcinoma

Stage

5-year survival rate

 

Stage 0

96%

Stage I

73%

Stage II

58%

Stage III/IV

36%

Adapted from American Cancer Society, www.cancer.org, revised 10-22-03


News
Age, Disease Stage May Decide Vaginal Cancer Outcome

Apr 25, 2014 - Women treated with laser vaporization for vaginal intraepithelial neoplasia may be at greater risk of recurrence if they are aged 48 or younger or if they were diagnosed with stage III cancer, compared to their older counterparts and those with less advanced disease, according to a study published online March 16 in Lasers in Surgery and Medicine.



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