Cancer of the Vagina

 

Vaginal and vulvar cancers are very rare. While all women are at risk for these cancers, very few will get them. the vagina is an elastic muscular tube comprising many mucosal folds. It extends from the cervix of the uterus to the hymenal ring and lies posterior to the bladder and anterior to the rectum. Its elasticity and dimensions vary depending on a woman's age, parity, previous surgeries, and hormonal status. Such characteristics can make proper examination (limited by pain or narrowness of the introitus) and identification of small malignant lesions difficult. Vaginal cancer occurs mainly in older women. The average age at the time of diagnosis is 67.

Although cancer of the vagina is more common in postmenopausal women, an increase in young women being diagnosed with primary vaginal cancer has been reported, especially in countries with a high HIV prevalence. This is associated with the persistence of high-risk HPV infection. The emphasis should be on primary prevention with prophylactic HPV vaccination.

 

Statistics of Vaginal cancer worldwide

1,368 women (0.6 per 100,000 women) were diagnosed with vaginal cancer.

431 women (0.2 per 100,000 women) died from vaginal cancer.

    Black women had the highest rate of getting vaginal cancer (0.9 per 100,000 women), followed by Hispanic women and White women (0.6) and Asian/Pacific Islander women (0.4). The rate for American Indian/Alaska Native women is suppressed.

    Black women had the highest rate of dying from vaginal cancer (0.3 per 100,000 women), followed by White women and Hispanic women (0.2). The rate for Asian/Pacific Islander women and American Indian/Alaska Native women is suppressed (too few people to calculate).

 

 

What Is Vaginal Cancer?

 

Vaginal cancer is a rare form of cancer that most often occurs in the cells lining your vagina. The vagina is a tube-like organ that connects your cervix (the lower part of your uterus) to your vulva (genitals). Despite its low incidence, vaginal cancer has many types.  According to the American Cancer Society, about 1 in every 1,100 women will develop vaginal cancer in their lifetime. An estimated 8,870 women in the United States are expected to be diagnosed with the disease in 2022.

 

Cancers that start in other parts of your body – like cervical cancer or uterine cancer – sometimes spread to your vagina. It’s less common for cancer to begin in your vagina, as with vaginal cancer.

Vaginal cancer types

There are different types of vaginal cancer. They’re named after the cells in your vagina where cancer starts.

 

    Squamous cell carcinoma begins in the flat cells that line your vagina, called squamous cells. Squamous cell carcinoma is the most common type of vaginal cancer. It accounts for nearly 90% of all cases.

    Adenocarcinoma begins in gland cells in your vagina. It’s most common in people over 50. Clear cell adenocarcinoma is the exception, often affecting people under 50 who were exposed to a drug called diethylstilbestrol (DES) when they were developing in the uterus.

    Melanoma begins in the cells that give your vagina its color (melanocytes). Vaginal melanomas are extremely rare.

    Sarcoma begins in the connective tissue and muscle tissue that make up your vaginal wall. Like vaginal melanomas, vaginal sarcomas are extremely rare.

There are different types of sarcoma. Rhabdomyosarcoma is the most common and mostly occurs in children. Leiomyosarcoma occurs most often in people over 50.

 

What causes vaginal cancer?

Your chances of getting vaginal cancer increase if:

 

    You’re over 60. Your risk of getting vaginal cancer increases with age. The average age that people get diagnosed with squamous cell carcinoma (the most common type) is 60. Occasionally, people younger than 60 develop vaginal cancer.

    You have human papillomavirus (HPV). HPV is a sexually transmitted virus that may increase your cervical cancer and vaginal cancer risk. Having multiple sex partners and being unvaccinated against HPV puts you at greater risk of infection.

    You’ve been diagnosed with vaginal intraepithelial neoplasia (VAIN). With VAIN, you have cells in your vaginal lining that aren’t normal, but they’re not cancer cells either. VAIN progresses to vaginal cancer in some people but not others. Researchers aren’t sure why. You’re more likely to develop VAIN if you have HPV.

    You’ve had cervical cancer or cervical dysplasia. It’s possible to develop vaginal cancer after being treated for cervical cancer. Abnormal cells in your cervix, or cervical dysplasia, may increase your risk of vaginal cancer.

    You’ve been exposed to diethylstilbestrol (DES). DES is a synthetic form of estrogen prescribed between 1940 and 1971 to prevent pregnancy complications. You’re at increased risk of developing adenocarcinoma if your birthing parent took DES during pregnancy and you were exposed.

    You smoke. Smoking doubles your risk of developing vaginal cancer.

 

Vaginal cancer symptoms

 

Early vaginal cancer may not cause any signs and symptoms. As it progresses, vaginal cancer may cause signs and symptoms such as:

 

Unusual vaginal bleeding, for example, after intercourse or after menopause

vaginal discharge that smells foul or has blood in it

painful sexual intercourse

Watery vaginal discharge

 A lump or mass in your vagina

Pain in the pelvis or low in the belly, and perineum (area between vagina and anus)

Back pain

change in bladder habits including pain, burning or trouble urinating, the need to urinate often, blood in the urine and urgent need to urinate

change in bowel habits including blood in the stool, constipation, and painful bowel movements

Swelling in the legs or groin

 

Having these symptoms does not always mean that you have cancer. In fact, these symptoms are more likely to be caused by something besides cancer, like an infection. The only way to know what’s causing these problems is to see a health care professional.

 

Diagnosing vaginal cancer

 

There are many tests used for diagnosing vaginal cancer. Not all tests described here will be used for every person. Your doctor may consider these factors when choosing a diagnostic test:

    symptoms that suggest vaginal cancer

    smoking

    exposure to diethylstilbestrol (DES) before birth

    vaginal, vulvar or cervical precancerous conditions and treatment

    cervical, vulvar or anal cancer

    HIV

 

A physical exam allows your doctor to look for any signs of vaginal cancer. During a physical exam, your doctor may:

 

    do a pelvic exam

    do a digital rectal exam (DRE) to feel for a lump or thickened area

    feel the lymph nodes in the groin and above the collarbone to see if they are swollen

 

    Pap test. During a Pap test, the doctor gently scrapes the outside of the cervix and vagina and takes a sample of cells for testing. During the test, you will usually feel some pressure as the doctor takes the sample of the cells, but there is usually no pain. Human papillomavirus (HPV) testing is usually part of the Pap test in people who are older than 30.

 

    Colposcopy. The doctor may do a colposcopy to check the vagina and cervix for any abnormalities, especially when Pap or HPV tests return abnormal results. A colposcopy is a special instrument, similar to a microscope, that magnifies the surface of the cervix and vagina. The colposcope gives the doctor a lighted, magnified view of the tissues of the vagina and the cervix. The colposcope is not inserted into the body. The examination is not painful, can be done in the doctor’s office, and has no side effects. This examination can be performed during pregnancy.

 

    Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. A pathologist analyzes the sample(s). A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease. The type of biopsy performed will depend on the location of the tissue being biopsied.

 

    If the biopsy indicates vaginal cancer, the doctor will refer the patient to a gynecologic oncologist, which is a doctor who specializes in treating vaginal cancer. The specialist may suggest imaging tests to see if cancer has spread beyond the vagina.

 

    Ultrasound. An ultrasound uses sound waves to create a picture of the internal organs.

 

    Endoscopy. An endoscopy allows the doctor to see inside the body with a thin, lighted, flexible tube called an endoscope. The person may be sedated as the tube is inserted through the mouth, anus, vagina, urethra, or a small surgical opening. Sedation is giving medication to become more relaxed, calm, or sleepy.

 

    Computed tomography (CT or CAT) scan. A CT scan takes pictures of the inside of the body using x-rays taken from different angles. A computer combines these pictures into a detailed, 3-dimensional image that shows any abnormalities or tumors. A CT scan can be used to measure the tumor’s size. Sometimes, a special dye called a contrast medium is given before the scan to provide better detail in the image. This dye can be injected into a patient’s vein or taken as a pill or liquid to swallow.

 

    Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A special dye called a contrast medium is given before the scan to create a clearer picture. This dye can be injected into a patient’s vein or taken as a pill or liquid to swallow.

 

    Positron emission tomography (PET) or PET-CT scan. A PET scan is usually combined with a CT scan (see above), called a PET-CT scan. However, you may hear your doctor refer to this procedure just as a PET scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive sugar substance is injected into the patient’s body. This sugar substance is taken up by cells that use the most energy. Because cancer tends to use energy actively, it absorbs more of the radioactive substance. Other conditions, such as infection or other causes of inflammation, can increase the uptake of sugar as well. However, the amount of radiation in the substance is too low to be harmful. A scanner then detects this substance to produce images of the inside of the body.

 

Endoscopic tests   These endoscopy procedures are not used often for women with vaginal cancer, but they may be needed in certain cases.

Proctosigmoidoscopy

 This test may be done if the vaginal cancer is large and/or in the part of the vagina next to the rectum and colon. Proctosigmoidoscopy looks at the rectum and part of the colon. It’s done to check for the spread of vaginal cancer to these organs. In this procedure, a thin, flexible, lighted tube is put into the rectum. The doctor can look closely and the inside of the rectum and the last part of the colon to look for cancer spread. Any areas that look suspicious will be biopsied. This test may be somewhat uncomfortable, but it should not be painful.

 

Cystoscopy may be recommended if vaginal cancer is large and/or is in the front wall of the vagina, near the bladder. This procedure allows the doctor to look at the inside of the bladder. It’s done to check for the spread of vaginal cancer to the bladder. It can be done in the doctor’s office or clinic. You might be given an intravenous (IV) drug to make you drowsy. A thin tube with a lens and light is put into the bladder through the urethra. If suspicious areas or growths are seen, a biopsy will be done.

 

Sentinel lymph node biopsy

The sentinel node is the first lymph node in a chain or cluster of lymph nodes that receives fluid from the area around a tumour. Cancer cells will most likely spread to these lymph nodes first. A sentinel lymph node biopsy removes the sentinel lymph node so it can be examined to see if it contains cancer cells. A sentinel lymph node biopsy may be done for vaginal melanoma.

 

Complete blood count (CBC)  A CBC measures the number and quality of white blood cells, red blood cells and platelets. A CBC is done to check for anemia from long-term, or chronic, vaginal bleeding. A CBC also gives doctors a baseline to compare future blood tests to during and after treatment.

 

Blood chemistry tests measure certain chemicals in the blood. They show how well certain organs are functioning and can help find abnormalities. Blood chemistry tests used to diagnose and stage vaginal cancer include the following:

 

    Blood urea nitrogen (BUN) and creatinine may be measured to check kidney function. Increased levels could mean that cancer has spread to the ureters or kidneys.

    Alanine aminotransferase (ALT), aspartate transaminase (AST) and alkaline phosphatase may be measured to check liver function. Increased levels could mean that cancer has spread to the liver.

 

Barium enema:  is an x-ray procedure that uses a contrast medium called barium sulphate. A contrast medium is a substance used in some diagnostic procedures to help parts of the body show up better on x-rays or other imaging tests. A barium enema is used to check if cancer has spread to the rectum.

 

Vaginal Cancer Treatment

 

The type of treatment your cancer care team recommends depends on the type of vaginal cancer you have, how far the cancer has spread, your overall health, and your preferences.

 

 

Surgery is a standard treatment option for both vaginal intraepithelial neoplasia (VaIN) and vaginal cancer.

 

The following types of surgery may be used to treat VaIN:

 

    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 a surface lesion such as a tumour.

    Wide local excision: A surgical procedure that takes out the cancer and some of the healthy tissue around it.

    Vaginectomy: Surgery to remove all or part of the vagina. Skin grafts from other parts of the body may be needed to reconstruct the vagina.

 

The following types of surgery may be used to treat vaginal cancer:

 

    Wide local excision: A surgical procedure that takes out the cancer and some of the healthy tissue around it.

    Vaginectomy: Surgery to remove all or part of the vagina. Skin grafts from other parts of the body may be needed to reconstruct the vagina.

    Total hysterectomy: Surgery to remove the uterus, including the cervix. If the uterus and cervix are taken out through the vagina, the operation is called a vaginal hysterectomy. If the uterus and cervix are taken out through a large incision (cut) in the abdomen, the operation is called a total abdominal hysterectomy. If the uterus and cervix are taken out through a small incision in the abdomen using a laparoscope, the operation is called a total laparoscopic hysterectomy.

Removal of the majority of the pelvic organs (pelvic exenteration). This extensive surgery may be an option if cancer has spread throughout your pelvic area or if your vaginal cancer has recurred.

 

During pelvic exenteration, the surgeon may remove many of the organs in your pelvic area, including your bladder, ovaries, uterus, vagina, rectum and the lower portion of your colon. Openings are created in your abdomen to allow urine (urostomy) and waste (colostomy) to exit your body and collect in ostomy bags.

 

If your vagina is completely removed, you may choose to undergo surgery to construct a new vagina. Surgeons use pieces of skin, sections of the intestine or flaps of muscle from other areas of your body to form a new vagina.

 

With some adjustments, a reconstructed vagina allows you to have vaginal intercourse. However, a reconstructed vagina isn't the same as your own vagina. For instance, a reconstructed vagina lacks natural lubrication and creates a different sensation when touched due to changes in surrounding nerves.

   

Stage 0 (also called VAIN 3 or carcinoma in situ [CIS])

 

The usual treatment options are laser vaporization, local excision, or intracavitary radiation (brachytherapy).

 

Topical therapy with 5-FU cream or imiquimod is also an option, but this often means treatment at least weekly for about 10 weeks.

 

If cancer comes back after these treatments, surgery (partial vaginectomy) might be needed.

 

Stage I

 Squamous cell cancers: Radiation therapy is used for most stage I vaginal cancers. If the cancer is less than 5 mm thick (about 3/16 inches), intracavitary radiation may be used alone. Interstitial radiation is an option for some tumours, but it’s not often used. For tumours that have grown more deeply, intracavitary radiation may be combined with external beam radiation.

 

Removing part or all of the vagina (partial or radical vaginectomy) might be needed depending on the size of the cancer and where it is in the vagina. Reconstructive surgery to create a new vagina after treatment of the cancer is an option if a large part of the vagina has been removed.

 

If the cancer is in the upper vagina, it may be treated with surgery, such as radical hysterectomy, bilateral radical pelvic lymph node removal, and/or radical or partial vaginectomy.

 

After radical partial or complete vaginectomy, radiation (external beam) may be used to treat cancer cells that might have spread to lymph nodes in the groin and/or pelvis.

 

Adenocarcinomas: For cancers in the upper part of the vagina, the treatment is surgery -- a radical hysterectomy, partial or radical vaginectomy, and removal of pelvic lymph nodes. This can be followed by reconstructive surgery if needed or desired. Both internal and external radiation therapy may be given as well.

 

For cancers lower down in the vagina, external beam radiation therapy may be used, along with either interstitial or intracavitary radiation therapy. The lymph nodes in the groin and/or pelvis are often treated with external beam radiation therapy.

 

Stage II

 

The usual treatment is radiation, using both brachytherapy and external beam radiation.

 Radical surgery (radical vaginectomy or pelvic exenteration) is an option for some women with stage II vaginal squamous cell cancer if it’s small and in the upper vagina. Radiation might be given after surgery. Surgery is also used to treat women who already had radiation therapy for cervical cancer and would have severe damage to normal tissues if more radiation was given.

 

If the tumour is in the lower third of the vagina, external radiation may be used to treat lymph nodes in the groin or pelvis.

 Chemotherapy (chemo) with radiation may also be used to treat stage II disease. 

Giving chemo to shrink the cancer before radical surgery may be helpful.

 

Stage III or IVA

 The usual treatment is radiation therapy, often with both brachytherapy and external beam radiation. Chemo might be combined with radiation to help it work better. Surgery is rarely used.

 

Stage IVB

 Since cancer has spread to distant sites, it can’t be cured. Radiation therapy to the vagina and pelvis might be used to ease symptoms and reduce bleeding. Chemo might also be given with the radiation. Chemo alone has not been shown to help women live longer. Because there’s no standard treatment for this stage, the best option is to enroll in a clinical trial.

Recurrent squamous cell cancer or adenocarcinoma of the vagina

 

If cancer comes back after treatment it's called recurrent cancer. If it comes back in the same place it was the first time, it's called a local recurrence. If it comes back in another part of the body, like the liver or lungs, it's called a distant recurrence.

 

A local recurrence of stage 1 or stage ll vaginal cancer may be treated with radical surgery (such as pelvic exenteration). If the cancer was treated with surgery before, radiation therapy is an option.

 

Surgery is the usual choice when cancer comes back after radiation therapy.

 

Higher-stage cancers are hard to treat when they recur. They usually can’t be cured. Care focuses mostly on relieving symptoms, although taking part in a clinical trial of new treatments may be helpful.

 

Vaginal Cancer: Follow-Up Care

 

Your follow-up care may include regular physical examinations, medical tests, or both. Doctors want to keep track of your recovery in the months and years ahead. Cancer rehabilitation may be recommended, and this could mean any of a wide range of services, such as physical therapy, occupational therapy, career counselling, pain management, nutritional planning, and/or emotional counselling. The goal of rehabilitation is to help people regain control over many aspects of their lives and remain as independent as possible

 

Although there are no specific guidelines, you should visit your doctor regularly for physical and pelvic examinations and a Pap test. Follow-up care is very important for people who have finished vaginal cancer treatment. The doctor may recommend other tests, including x-rays, CT scans, ultrasounds, or MRI scans.

 

Follow-up visits for cervical cancer are usually scheduled:

    every 3 to 4 months for the first 2 years after initial treatment

    every 6 to 12 months for the next 3 years (years 3 to 5)

    once a year after 5 years

Don’t wait until your next scheduled appointment to report any new symptoms and symptoms that don’t go away. Tell your healthcare team if you have:

 

    pain in the pelvis, hips, back or legs

    bleeding or discharge from the vagina

    changes in bowel habits or bladder function

    unexplained weight loss

    a cough that doesn’t go away

 

The chance that cervical cancer will come back (recur) is greatest within 2 to 3 years, so you will need close follow-up during this time.

 

Managing long-term and late side effects

 

Most people expect to have side effects when receiving treatment. However, it is often surprising to survivors that some side effects may linger beyond the treatment period. These are called long-term side effects. Other side effects called late effects may develop months or even years after treatment has ended. Long-term and late effects can include both physical and emotional changes.

 

Talk with your doctor about your risk of developing such side effects based on your diagnosis, your individual treatment plan, and your overall health. If you had a treatment known to cause specific late effects, you may have certain physical examinations, scans, or blood tests to help find and manage them.

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