Vulvar Cancer

Vulvar Cancer - What is Vulvar Cancer?

Vulvar cancer or the cancer of the vulva is a gynaecological cancer that is on the rise with around 1,000 new cases being diagnosed each year in the UK.

However, that said, vulvar cancer is rare and accounts for less than 1% of all cancer cases in the UK. North America has the highest incidence and Asia the lowest rates of vulvar cancer.

Anatomy of the vulva

The vulva comprises of the external sex organs of a woman. It is made up of the opening of the vagina, the external and internal lips covering the vagina called labia majora and minora respectively.

These shield the opening of the urethra, the clitoris (a small fleshy bit that is located over the urethral opening) and the vaginal opening. In 75% cases the cancer begins in the labia. (1-5)

Symptoms of vulvar cancer

Common symptoms of the condition are development of a small mass, lump or wart-like growth over the vulva. This may or may not be accompanied by persistent itchiness, pain on urination or passing blood or blood stained discharge.

Types of vulvar cancer

Vulvar cancer is divided into various types. The types are classified based on the cells that are primarily affected.

The commonest type of vulvar cancer is squamous cell carcinoma, which accounts for over 90% all cases of vulvar cancer. Here the cancer begins in the skin and outer layers.

The second common type of vulvar cancer is called vulval melanoma that comprises of 4% of all cases. These develop in the pigment and color producing cells of the skin.

Rarer types of vulvar cancer includes adenocarcinoma that affects the cells that line the glands in the vulva, verrucous carcinoma that is a slow growing cancer that begins as a wart and a sarcoma that develops in tissue such as muscle or fat under the skin.

Who is at risk of vulvar cancer?

Women who are most at risk of vulvar cancer are those over the age of 65. Women with lichen sclerosis – a skin condition that is not cancerous are also at a higher risk. In fact, of every 5 cases of vulvar cancer, advanced age and history of lichen sclerosis is present in at least 3 to 4.

The other cases are made up of women who have not yet reached menopause. Smoking poses another major risk factor. Women with a persistent infection with specific high-risk strains of the human papilloma virus (HPV) known as HPV 16 that may lead to cervical cancer are at risk of vulvar cancer as well.

Vulval intraepithelial neoplasia (VIN) is a condition where skin cells around the vulva show changes under the microscope that are deemed “pre-cancerous” or have a potential to turn into cancer. HPV infection and VIN are responsible for the majority of cases in younger women.

Treatment of vulvar cancer

Treatment mainly involves surgery. Surgery helps to remove the cancer as much as possible before starting therapy with other modalities such as radiotherapy and chemotherapy. Most women who undergo treatment for vulvar cancer recover fully.

It is seen that 70% of people with vulvar cancer survive at least five years after diagnosis with appropriate and timely therapy. Some live longer while others may even be cured.

Squamous cell carcinomas if diagnosed early have the best outcomes with 90% five year survival rate.

Causes of Vulvar Cancer

Vulvar cancer is a rare form of cancer affecting the vulva and the external genital organs in women. It accounts for less than 1% of all cancer cases in the UK.

Who does vulvar cancer affect?

The cancer rarely affects the young and most commonly affects women over 65.

How common is vulvar cancer?

The lifetime risk in the UK is 1 in 316 women.

In the United States, vulvar cancer accounts for about 4% of cancers of the female reproductive organs and 0.6% of all cancers in women. In the US, women have a 1 in 406 chance of developing vulvar cancer during their lifetime. (1-5)

Types of vulvar cancer

Most vulvar cancers are of squamous cell carcinoma type – around 75 to 85%. The rest are of various other types like melanoma, adenocarcinoma etc.

In half of the cases the labia majora – the external lips of the vulva is affected. The inner lips or labia minora is affected in nearly 205 cases. In small number of cases the glands and the clitoris is affected.

How does vulvar cancer begin?

Cancer starts when the blue print within the cells called the DNA and genes are altered. This leads to uncontrolled multiplication of the affected cells till they invade surrounding cells.

How does vulvar cancer spread?

Vulvar cancer spreads in three major ways. It spreads out of the vulvar tissue into surrounding parts of the body like the urethral opening and the vagina.

It may also spread via the lymphatic channels and lymph nodes to affect the whole body. The first lymph nodes that are invaded are the nearest ones in the groin.

The cancer may also spread via blood vessels to large organs like liver, lungs and brain. Cancer that has spread to other parts of the body is known as metastatic cancer.

Risk factors for vulvar cancer

Exact cause of vulvar cancer is unknown. However some risk factors may play a role in this cancer. Risk factors of vulvar cancer include:

  • Advancing age - Two thirds of the cases of vulvar cancer are seen in women over 65 and half of the cases in women over 75.
  • Long term infection with human papilloma virus (HPV) - Specific strains such as HPV 16 are responsible for cervical cancer. They are also implicated in vulvar cancer.  The HPV virus is spread during unprotected sexual intercourse, including anal and oral sex.
  • Vulvar intraepithelial neoplasia (VIN) - This is a pre-cancerous condition where the cells are altered in their function and appearance but not yet cancerous. There may be symptoms like itchiness, burning sensation on urination and raised red or white patches of skin.  VIN may be of two types - usual VIN or undifferentiated VIN seen in women between 35 and 55. It is due to HPV infection. Differentiated VIN or dVIN is a rare type seen in women between 55 and 85. It is associated with skin conditions of the vulva. VIN is treated using a cream called Imiquimod that kills abnormal cells.
  • Non-cancerous skin conditions of the vulva - Women who have non-cancerous skin conditions of the vulva like lichen sclerosis are at an increased risk of vulvar cancer. Around 1 in 50 women with these skin lesions go on to develop vulvar cancer.

Other risk factors for vulvar cancer

Other risk factors associated with vulvar cancer include:

  • Smoking
  • Those infected with human immunodeficiency virus (HIV) are also at a raised risk of HPV infection and in turn raises risk of vulvar cancer.
  • Those with abnormal looking moles or lesions in their vulva are at a higher risk of the mole turning to vulvar cancers like melanoma.
  • Other sexually transmitted disease, having multiple sexual partners, never having had children etc. are other risk factors of vulvar cancer.

Symptoms of Vulvar Cancer

Vulvar cancer is a relatively less common type of cancer affecting the external female genitalia. The cancer commonly is of the squamous cell variety and affects the labia majora or minora.

Common symptoms of vulvar cancer

The common symptom of vulvar cancer is a continuous itch.

Distinctive symptoms of vulvar cancer

Some of the other more distinctive symptoms of the condition include (1-5);

  • Long term itch over the vulva. This is a nonspecific complaint that may be seen in various conditions and is usually not a diagnostic symptom of vulvar cancer.
  • Some women may develop a mass, swelling or lump over the vulva. This is usually over the labia or near it.
  • There may be pain and discomfort in the vulva especially on urination or during sexual intercourse.
  • There are raised, reddish or whitish or even blackened skin patches.
  • There may be bleeding or blood stained discharge from the vulva or vagina. This is usually unrelated to menstrual periods.
  • There may be a mole or wart over the vulva that has recently changed in shape, size or color.
  • Some women may develop lesions with ulcers over the vulva. This is an open sore that persists for more than a month.
  • If the lesion is advanced and spreading it may affect the nearest lymph nodes of the groin. These lymph nodes may appear swollen and rubbery.

Spreading of vulvar cancer

Spread may occur to the vagina, urethra and anal canal as well. This called local spread.

If the cancer has spread to the urinary tract there may be difficulty in urinating. If the cancer has spread to the anal canal there may be problems with bowel motions etc.

Around 5% cases show a more distant spread. This may involve major organs like lungs, liver and brain. The consequences are usually life threatening.

Spread to the liver manifests as jaundice and that to the lungs as fluid collection around the lungs and breathlessness. The cancer can also spread to bones causing bone pain and easy fractures.

Symptoms of Vulvar intraepithelial neoplasia (VIN)

Vulvar intraepithelial neoplasia (VIN) is the precancerous condition that in 80% cases may lead to vulvar cancer.

Symptoms of this condition include itching and soreness, burning or tingling feeling over the affected area.

There are areas of redness and whitish patches that are raised and warty in feel and appearance. In many cases there may be no symptoms at all.

History of conditions prior to Vulvar cancer

There may be a history of Human Papilloma virus infection (HPV infection) or a noncancerous skin condition like lichen sclerosus.

Symptoms of specific cancers

  • Verrucous carcinoma - This is a subtype of cancer that appears as cauliflower-like growths similar to herpes infected genital warts.
  • Vulvar melanoma - This commonly affects the cells that produce the pigment melanin producing cells of the vulvar skin. This appears as a darkly colored growth or a change in a mole. The mole is characteristically detected by the ABCD rule. A is for asymmetry, B is for irregular Borders, C is for abnormal color change. Moles are usually brown or tan or black. Changes in color are a warning sign. D is for Diameter. An affected mole usually changes in its size as well.
  • Bartholin gland cancer - This usually shows up as a lump on either side of the vaginal opening.

Diagnosis of Vulvar Cancer

Vulvar cancer is diagnosed with a complete medical history, physical examination followed by laboratory and imaging studies.

Most cancers need to be staged into various stages and classes which determine their method of treatment and also determine the chances of cure and survival after therapy.

Detailed medical history

Diseases in the past, especially lichen sclerosus as well as Human Papilloma virus (HPV) infection, in the past is significant.

Diagnosis of Vulvar intraepithelial neoplasia (VIN) is also important since around 80% of women with VIN go on to develop vulvar cancer.

Physical examination

A detailed physical examination is performed. Pelvic and vulvar examination helps determine the site and extent of local spread of the cancer. Presence of lumps, moles, warts, raised patches etc. is noted in detail.

Surrounding structures like anal canal and urethra are examined and signs of spread are determined. Local lymph nodes, and other major organs are also examined to detect spread of the disease.

Colposcopy and biopsy

This is a common first step in detailed diagnosis. An instrument called colposcope is a small tube like structure with a light and camera at its end. It helps to visualize the cancer and take small amount of tissue samples. This is called a tissue biopsy.

Before a biopsy is taken the area is smeared with a numbing local anesthetic cream. There may be a slight bleeding or soreness of the biopsied area.

Biopsies may also be taken from swollen groin lymph nodes to determine the extent of spread of the cancer. This can be done using a needle called Fine needle aspiration cytology (FNAC).

Or in some cases a short surgery to inspect the lymph node is performed.

In all cases of biopsy the sample is stained with a special dye and examined under the microscope to see if there is cancer and the type of cancer and the type of cancer.

Further tests

Once cancer is confirmed with biopsy further tests are suggested. Next step is to perform a cystoscopy. This uses a thin long tube with a camera on its tip that is inserted into the urinary bladder to check if there is a spread into the bladder.

Biopsy samples are taken if there is a suspected lesion. Proctoscopy is like cystoscopy and checks for cancer spreads to the anal canal and rectum.

Other scans

  • A CT scan is suggested to look at a detailed picture within the body. This shows the extent of spread of the cancer.
  • A MRI scan is another imaging study that shows the parts of the body in greater details that CT scan.
  • A chest X ray reveals spread of the cancer to the lungs.
· A Positron emission tomography (PET) scan – this uses a glucose molecule with a radioactive atom. Since cancers hoard up glucose more than other cells the radioactivity tends to concentrate in the cancer. This can be detected by a special camera. This test also helps detect spread of the cancer.

Staging the cancer

The tests are conducted to stage the cancer. Staging reveals the extent of spread of the cancer.

If the stage is lower, it means that the cancer has not spread extensively and there is a chance of a complete cure.

Stages for vulvar cancer

Stages for vulvar cancer are:

  • Stage 1 – the cancer is confined to the vulva. Stage 1A is when the cancer is less than 2cm in size and 1B is when the cancer is more than 2cm in size.
  • Stage 2 – This stage indicates that the cancer has spread to the nearby structures like the vagina, anus and the urethra but the nearest lymph nodes are not affected.
  • Stage 3 – This stage indicates that the cancer has spread to the nearby lymph nodes.
  • Stage 4 - indicates that the cancer has spread to other parts of the body (1-4)