Vulvar cancer, or vulval cancer, is a relatively rare type of cancer that affects the vulva, the external genital organs that protect a woman's reproductive system.

It is most likely to appear in the outer vaginal lips. Typical symptoms include a lump, itching, and bleeding.

Vulvar cancer accounts for around 0.6 percent of all cancers in women. The American Cancer Society predict that in 2017 there will be about 6,020 diagnoses of vulvar cancers in the United States, and that 1,150 women will die of vulvar cancer.

Fast facts on vulvar cancer:

Here are some key points about the vulvar cancer. More detail is in the main article.

  • Vulvar cancer affects the external genital organs of a woman, most commonly the outer lips of the vagina.
  • Symptoms include a lump, itching, and bleeding, and with some types discoloration of the skin and pain.
  • Early diagnosis increases the chance of successful treatment dramatically, but without treatment, cancer can spread to other parts of the body.
  • Regularly attending smear tests and checking for changes to the vaginal lips can help diagnose vulvar cancer in the early stages.
  • Avoiding smoking and unprotected sex can reduce the risk.

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Symptoms of vulvar cancer include itching and pain.

The vulva includes the labia majora, mons pubis, labia minora, clitoris, the vestibule of the vagina, the bulb of the vestibule, greater and lesser vestibular glands, and vaginal orifice.

Vulvar cancer most commonly affects the outer lips of the vagina.

Cancer that originates in the vulva is called primary vulvar cancer. If it starts in another part of the body and then spreads to the vulva, it is called secondary vulvar cancer.

There are several types of vulvar cancer.

Squamous cell carcinoma affects the flat, outer layers of skin. In medicine, the word squamous refers to flat cells that look like fish scales. About 90 percent of all vulvar cancers are squamous cell carcinomas. It takes several years for noticeable symptoms to develop.

Vulvar melanoma accounts for about 5 percent of all vulvar cancers. A melanoma presents as a dark patch of discoloration. There is a high risk of this type of cancer spreading to other parts of the body, a process known as metastasis. It may affect younger women.

Adenocarcinoma originates in glandular tissue, and in this case, the cells line the glands in the vulva. It accounts for a very small proportion of vulvar cancers.

Sarcoma originates in the connective tissue. Most cancers of this type are malignant. It is rare.

Verrucous carcinoma is a subtype of the squamous cell cancer, and it tends to appear as a slowly growing wart.

The outlook is normally good if diagnosis happens in the early stages, before the cancer spreads, and if the woman receives prompt and appropriate treatment.

The first sign is usually a lump or ulceration, possibly with itching, irritation, or bleeding.

Sometimes, a woman may not seek medical help at once due to embarrassment, but an early diagnosis will improve the outlook.

Most typical symptoms include:

  • painful sexual intercourse
  • bleeding
  • pain and burning
  • dark discoloration in cases of melanoma
  • painful urination
  • persistent itching
  • rawness and sensitivity
  • wart-like growths
  • thickened skin
  • ulceration

Different types of vulvar cancer may have different symptoms, and in some cases, there may be no noticeable symptoms. Any changes that take place should be checked with a doctor.

Cancer happens when cell growth is out of control.

Most cancers harm the body when damaged cells divide uncontrollably to form lumps or masses of tissue, or tumors. Tumors can grow and affect body function. A benign tumor stays in one place and does not spread, but a malignant tumor spreads and causes further damage.

Malignancy occurs when two things happen:

  1. a cancerous cell manages to move throughout the body using the blood or lymph systems, destroying healthy tissue via a process called invasion.
  2. the cell divides and grows through a process called angiogenesis, making new blood vessels to feed itself.

Without treatment, cancer can grow and spread to other parts of the body. This is called metastasis. If it enters the lymphatic system, it can reach other parts of the body, including vital organs.

Experts do not know exactly why cells start to grow too fast, but certain risk factors increase the chance of developing the disease.

Age: Over half of all cases are in women aged over 70 years, and fewer than 1 in 5 occur before the age of 50 years.

Human papilloma virus (HPV): Women infected with HPV have a higher risk of developing vulval cancer.

Vulvar intraepithelial neoplasia (VIN): This is a general term for a precancerous state, in which certain cells within the vulvar epithelium have a range of low-grade carcinoma. Women with VIN have a significantly higher risk of developing vulvar cancer.

Lichen sclerosus et atrophicus (LSA): This causes the skin to become thick and itchy, and it may increase the susceptibility to vulvar cancer slightly.

Melanoma: A personal or family history of melanoma in other parts of the body increases the risk of vulvar cancer.

Sexually transmitted infections (STIs): Women with a higher level of antibodies to the herpes simplex virus type 2 appear to have a higher risk of vulvar cancer.

Smoking: studies suggest that women who smoke regularly have a three to six times increased risk of vulvar cancer. If the regular smoker also has the HPV infection, the risk is higher still.

Kidney transplant: a kidney transplant appears to increase the chances of developing vulvar cancer. This may be due to the use of immunosuppressant drugs. These drugs are used for the rest of a patient's life after a transplant to keep the body from rejecting the organ.

Human immunodeficiency virus (HIV): people with HIV or AIDS are more susceptible to HPV infection.

Other risk factors include having systemic lupus erythematosus, also known as SLE or lupus, having psoriasis, or having radiotherapy for womb cancer.

The doctor will carry out a gynecological evaluation, which includes checking the vulva.

If there is an ulceration, lump, or a mass that looks suspicious, a biopsy is required.

The examination should include the perineal area, including the areas around the clitoris and urethra. The doctor should also palpate the Bartholin's glands. Anesthesia may be used.

Depending on the results of the biopsy, there may be further tests:

  • Cystoscopy: the bladder is examined to determine whether the cancer has spread to that area.
  • Proctoscopy: the rectum is examined to check whether the cancer has spread to the rectal wall.
  • Imaging scans: these can help the doctor determine whether the cancer has spread, and if so, where to. An MRI or CT scan may be used. X-rays may be used to determine whether the cancer has reached the lungs.

If a biopsy confirms the presence of vulvar cancer, the doctor will stage it with the help of imaging scans.

Staging

There are different ways of staging cancer.

The four-stage system is as follows:

  • Stage 0, or carcinoma in situ: The cancer is only on the surface of the skin.
  • Stage 1: The cancer is limited to the vulva or perineum and is up to 2 centimeters in size.
  • Stage 2: The same as stage 1, but the tumor is at least 2 centimeters in size.
  • Stage 3: The cancer has reached nearby tissue, such as the anus or vagina, and it may have reached the lymph nodes.
  • Stage 4: The cancer has reached the lymph nodes on both sides of the groin, and it may have reached the bowel, the bladder, or the urethra, the passage through which urine leaves the body.

It is important to seek early diagnosis and treatment, to prevent the spread of cancer.

The types of treatment normally used for vulvar cancer are surgery, chemotherapy, radiation therapy, and biologic therapy.

Surgery is the main way to treat vulvar cancer. Treatment aims to remove the cancer while leaving sexual function intact.

If diagnosis occurs in the early stages of the cancer, limited surgery is required.

At the later stages, and if the cancer has spread to nearby organs, such as the urethra, vagina or rectum, surgery will be more extensive.

Types of surgery include:

  • Laser surgery: This uses a laser beam as a knife, to remove lesions.
  • Excision: The surgeon attempts to remove all the cancer and some healthy tissue around it.
  • Skinning vulvectomy: The surgeon removes the top layer of skin, where the cancer is located. A skin graft from another part of the body can be used to replace what was lost.
  • Radical vulvectomy: The surgeon removes the whole vulva, including the clitoris, vaginal lips, the opening to the vagina, and usually the nearby lymph nodes as well

Radiation therapy can shrink deep lesions or tumors before surgery, so they will be easier to remove. It can also treat lymph nodes. It can be used to relieve symptoms and improve quality of life. How it is used depends on which stage the cancer has reached.

Chemotherapy is often used with radiotherapy as part of palliative care. It may be used on the skin, as a cream or lotion, but the method will depend on whether and how far the cancer has spread.

Reconstructive surgery may be possible, depending on how much tissue is removed. Plastic surgery reconstruction can involve skin flaps, and a skin graft is sometimes possible.

Biologic therapy is a kind of immunotherapy. It uses either synthetic or natural substances to help the body defend itself against cancer. Imiquimod is an example. It may be applied topically, as a cream, to treat vulvar cancer.

Up to 24 percent of vulvar cancers will eventually come back. It is important to attend follow-up visits.

Measures that can reduce the risk of developing vulvar cancer include:

  • practicing safe sex
  • attending scheduled cervical smear tests
  • having the HPV vaccination
  • not smoking

There is no standard screening for vulvar cancer, but women should carry out checkups as their doctor recommends, and be aware of any changes to their body. The outlook is better with early diagnosis.

If vulvar cancer is diagnosed in the local stage, when it is still in a limited area, the relative chance of living for at least 5 years after diagnosis is 86 percent.

If it has spread to nearby lymph nodes or tissues, the patient has a 54-percent chance of living for at least 5 years. If diagnosis happens when the cancer has already reached more distant organs, the chance of surviving at least 5 more years is 16 percent.

It is important to attend regular pap tests and to look out for any unusual changes, because finding a cancer early increases the chance of a good outcome.