Vulvar Cancer (Vulval Cancer)

What Is Vulvar Cancer (Vulval Cancer)? What Causes Vulvar Cancer?

Vulvar cancer, or vulval cancer is a cancer in the vulva and accounts for approximately 1 in every 25 female cancers - most health authorities worldwide say it is a rare type of cancer. The vulva refers to the external genital organs of a female, and includes the labia majora, mons pubis, labia minora, clitoris, bulb of the vestibule, vestibule of the vagina, greater and lesser vestibular glands, and vaginal orifice.

Vulval cancer generally affects older women. According to the NIH (National Institutes of Health), USA, approximately 3,740 new cases of vulvar cancer were diagnosed in 2006, in the same year about 880 women died of the disease. The NHS (National Health Service), UK informs that about 1,000 patients are diagnosed with this type of cancer annually.

Typical symptoms include itching, bleeding and pain.

If the cancer originated in the vulva it is called primary vulvar cancer. If the cancer originated in another part of the body and then spread to the vulva it is called secondary vulvar cancer.

Types of vulval cancer

  • Squamous cell carcinoma - in medicine, the word squamous refers to flat cells that look like fish scales - in this case it refers to the outer layers of skin (which are flat). About 90% of all vulvar cancers are of this type. The cancer takes several years to develop into noticeable symptoms.
  • Vulvar melanoma - this type makes up about 5% of all vulval cancers. The vulval melanoma behaves like melanoma in other locations and may affect younger women. With this type of cancer there is a high risk of metastasis (spreading into other parts of the body)
  • Adenocarcinoma - this type of cancer originates in glandular tissue, which in this case are the cells that line the glands in the vulva. A very small proportion of vulval cancers are of this type.
  • Sarcoma - this type of cancer originates in the connective tissue. Most cancers of this type are malignant. This is an extremely rare type of vulval cancer.
  • Verrucous carcinoma - a subtype of the squamous cell cancer and tends to appear as a slowly growing wart.
If the cancer is localized - has not metastasized (spread to other parts of the body) - the prognosis is generally good if the patient receives prompt and proper treatment.

What are the signs and symptoms of vulvar cancer?

A symptom is something the patient feels and reports, while a sign is something other people, such as the doctor detect. For example, pain may be a symptom while a rash may be a sign.

Usually there is some kind of lump or ulceration (open skin sore); the area will typically itch, irritate and sometimes bleed.

Sometimes, because of modesty or embarrassment the patient may not seek medical help as soon as symptoms appear.
Most typical symptoms include:
  • Dyspareunia - painful sexual intercourse
  • Bleeding
  • Burning
  • Dark discoloration in cases of melanoma
  • Dysuria - painful urination
  • Persistent itching
  • The area may be sensitive and raw
  • Wart-like growths

What causes vulvar cancer?

Cancer is a class of diseases characterized by out-of-control cell growth. Cancer harms the body when damaged cells divide uncontrollably to form lumps or masses of tissue called tumors (except in the case of leukemia where cancer prohibits normal blood function by abnormal cell division in the blood stream). Tumors can grow and interfere with and alter body function. Tumors that stay in one spot and demonstrate limited growth are generally considered to be benign, dangerous cancer that spreads is malignant.

More dangerous, or malignant, tumors form when two things occur: 1. a cancerous cell manages to move throughout the body using the blood or lymph systems, destroying healthy tissue in a process called invasion. 2. that cell manages to divide and grow, making new blood vessels to feed itself in a process called angiogenesis.

If the patient with malignant cancer receives no treatment, it can grow and spread to other parts of the body (metastasis). As soon as the cancer gets into the lymphatic system it can more effectively reach other parts of the body, including vital organs.

Experts do not know exactly why the runaway growth of cancer cells starts. We do know, however, that certain risk factors increase the probability of developing the disease. These include:
  • 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 vulval cancer.
  • Sexually transmitted infections - women with antibodies to the herpes simplex virus type 2 have been linked to a higher increase of vulval cancer.
  • Systemic lupus erythematosus- one study showed a three-fold increase in vulval cancer risk.
  • Smoking- studies have revealed an association between regular smoking and vulval cancer, ranging from a three-fold to six-fold increase. If the regular smoker also has HPV infection, the risk is much higher still.
  • Kidney transplant - women who have had a kidney transplant have a much higher risk of developing vulval cancer. Doctors believe it is due to the immunosuppressant drugs (to stop organ rejection by the body) the patient has to take for life.
  • Human immunodeficiency virus (HIV)- People with HIV/AIDS are more susceptible to HPV infection.
  • Psoriasis - women with psoriasis have a significantly higher risk of developing vulval cancer.

How is vulvar cancer diagnosed?

The doctor will carry out a gynecologic evaluation, which includes checking the vulva - this may reveal the presence of an ulceration, lump, or a mass. If a lesion looks suspicious a biopsy is required. The examination of the vulva should include the perineal area, including areas around the clitoris and urethra, as well as the Bartholin's glands (palpation). If necessary anesthesia can be used for a more thorough examination.

Depending on the results of the biopsy, the doctor may order 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: Imaging scans 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.

Staging of vulvar cancer

If a biopsy confirms the presence of vulval cancer, the doctor will stage it with the help of imaging (CT or MRI). Staging guidelines used in the UK:
  • Stage 0 - known as carcinoma in situ; the cancer only exists on the surface of the skin.
  • Stage 1 - the cancer is limited to the vulva or perineum and is up to 2cm in size.
  • Stage 2 - same as 1, but the tumor is at least 2cm in size.
  • Stage 3 - the cancer has reached nearby tissue, such as the anus or vagina. It may also have reached the lymph nodes.
  • Stage 4 - the cancer has reached lymph nodes on both sides (of the groin). It may also have reached the urethra (where urine comes out of), the bowel or the bladder.

What are the treatment options for vulvar cancer?

Surgery is the mainstay of therapy for vulval cancer and includes the use of a radical vulvectomy, where the entire vulva is surgically removed, and possibly the removal of lymph nodes as well. If the cancer has spread to adjacent organs, such as the urethra, vagina or rectum, the surgery will be more extensive. In cases of early vulval cancer the procedure is less radical and disfiguring.
  • Laser surgery - an option during the early stages of the cancer.
  • Excision - the surgeon attempts to remove all of the cancer and some healthy tissue around it.
  • Skinning vulvectomy - the top layer of skin where the cancer is located is surgically removed. Skin from another part of the body can be used to replace what was lost (skin graft)
  • Radical vulvectomy - the whole vulva is surgically removed, including the clitoris, vaginal lips and the opening to the vagina. Usually includes nearby lymph nodes as well.
Radiation therapy - if lesions (tumors) are very deep, local radiotherapy may be used before surgery to shrink them - this makes it easier for the surgeon to get them out cleanly. Radiotherapy may also be used to treat lymph nodes.

Chemotherapy - often used with radiotherapy as part of palliative care.

Reconstructive surgery - sometimes the area can be reconstructed; this depends on how much tissue was removed. Plastic surgery reconstruction involving skin-flaps can be performed. Skin can sometimes be grafted from another part of the body.

Follow-up - in approximately 10% of cases, the cancer eventually comes back. The patient should make sure she attends her follow-up visits according to her doctor's instructions.


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