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. It is most likely to affect the outer vaginal lips.
Vulvar cancer accounts for around 0.6 percent of all cancers in women.
The American Cancer Society predicts that in 2017 there will be about 6,020 diagnoses of vulvar cancers in the United States. Of these diagnoses, 1,150 women will die of vulvar cancer.
Typical symptoms include itching, bleeding, and pain.
If the cancer originates in the vulva, it 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.
Contents of this article:
Types of vulvar cancer
Cancer cells damage the body after dividing and replicating.
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. This type accounts for a very small proportion of vulvar cancers.
Sarcoma originates in the connective tissue. Most cancers of this type are malignant. This is a rare type of vulvar cancer.
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 the cancer is localized at diagnosis, meaning that it has not spread, and if the woman receives prompt and appropriate treatment.
Signs and symptoms
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
- Dark discoloration in cases of melanoma
- Painful urination
- Persistent itching
- Rawness and sensitivity
- Wart-like growths
- Thickened skin
Different types of vulvar cancer may have different symptoms, but if any changes take place, it is a good idea to see 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 called tumors. Tumors can grow and affect body function. Tumors that stay in one spot and demonstrate limited growth are generally considered to be benign. Cancer that spreads is dangerous, and it is known as malignant.
More dangerous, or malignant, tumors form when two things occur:
Smoking is one of many factors that can increase the risk of vulvar cancer.
- A cancerous cell manages to move throughout the body using the blood or lymph systems, destroying healthy tissue via a process called invasion.
- The cell divides and grows through a process called angiogenesis, making new blood vessels to feed itself.
If a patient with cancer receives no treatment, it can grow and spread to other parts of the body. This is called metastasis. If the cancer 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 probability of developing the disease.
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.
Sexually transmitted infections: women with a higher level of antibodies to the herpes simplex virus type 2 appear to have a higher risk of vulvar cancer.
Systemic lupus erythematosus: one study showed that this could increase the risk of vulvar cancer threefold.
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.
Psoriasis: Women with psoriasis have a significantly higher risk of developing vulvar cancer.
Diagnosis, treatment, and prevention
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. If necessary, anesthesia can be used for a more thorough examination.
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 (CT or MRI).
Staging of vulvar cancer
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
The American Cancer Institute's SEER program describes the stages of vulvar cancer as follows:
- Local: cancer only affects the vulva, as in stages I and II.
- Regional: the cancer has spread to nearby lymph nodes or tissues, but not to other organs, as in stage 3 and the beginning of stage 4.
- Distant: the cancer affects distant organs or tissues, as in later Stage 4.
It is estimated that if vulvar cancer is diagnosed in the local stage, the relative chance of living for at least 5 years after diagnosis is 86 percent. If cancer is diagnosed at the regional stage, there is 54 percent chance of living for at least 5 years, and if diagnosis happens at the distant stage, the rate is 16 percent.
Surgery is the main way to treat vulvar cancer. Treatment aims to remove the cancer while leaving sexual function intact.
Radiotherapy is one method used by oncologists to treat vulvar cancer.
If diagnosis occurs in the early stages of the cancer, limited surgery is required.
At the later stages, more extensive treatment will be needed. If the cancer has spread to nearby organs, such as the urethra, vagina or rectum, the surgery will be more extensive.
Types of surgery include:
- Laser surgery uses a laser beam as a knife, to remove lesions.
- Excision is where the surgeon attempts to remove all the cancer and some healthy tissue around it.
- Skinning vulvectomy involves removing the top layer of skin on which the cancer is located. Skin from another part of the body can be used to replace what was lost in a skin graft.
- Radical vulvectomy, where 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.
Chemotherapy is often used with radiotherapy as part of palliative care.
Reconstructive surgery may be possible depending on how much tissue is removed. Plastic surgery reconstruction can involve skin flaps, and skin can sometimes be grafted from another part of the body.
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.