What is Vulvar Cancer?
Cancer occurs when abnormal tissue cells reproduce uncontrollably. Cancer can develop anywhere in the body, and the symptoms and treatment depend on the type of cancer and its location. There are various types of cancer that can affect the female reproductive organs, including vulvar cancer.
Vulvar cancer is a cancer of the vulva, or a female’s external genitals. The vulva includes the inner and outer lips of the vagina, the clitoris, and the opening of the vagina, which is called the introitus. Glands near the vaginal opening are also part of the vulva. Vulvar cancer typically affects the outer lips of the vagina, but other parts of the vulva may also be affected, especially as the cancer enlarges.
This type of cancer usually develops slowly. It often begins as vulvar intraepithelial neoplasia, which occurs when healthy skin cells around the vulva undergo abnormal changes. Without treatment, the abnormal cells can turn into cancer.
Types of Vulvar Cancer
There are several main forms of this disease.
Squamous cell carcinoma. This is the most common type. It starts in your skin cells. It might be linked to the human papillomavirus (HPV), especially in younger women. A subtype called verrucous carcinoma grows slowly and can look like a wart.
Adenocarcinoma. This type usually starts in cells in the Bartholin’s glands just inside the opening of your vagina. This is also called Bartholin gland cancer. It can look like a cyst. It also can form in sweat glands in the skin of your vulva or in the top layer of vulvar skin, which is called Paget’s disease.
Melanoma. This type forms in cells that make pigment, or skin color. You’re more likely to get it on skin that’s exposed to the sun, but it sometimes forms on your vulva.
Sarcoma. This starts in bone, muscle, or connective tissue cells. It can happen at any age, including in childhood.
Basal cell carcinoma. This is the most common type of skin cancer. It usually appears on skin that’s exposed to the sun, rarely on the vulva.
Pathology of Vulvar Cancer
About 90% of vulvar cancers are squamous cell carcinomas; about 5% are melanomas. Others include adenocarcinomas and transitional cell, adenoid cystic, and adenosquamous carcinomas; all may originate in Bartholin glands. Sarcomas and basal cell carcinomas with underlying adenocarcinoma also occur.
Vulvar cancer may spread as follows:
- By direct extension (eg, into the urethra, bladder, vagina, perineum, anus, or rectum)
- Hematogenously
- To the inguinal lymph nodes
- From the inguinal lymph nodes to the pelvic and para-aortic lymph nodes
Causes
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:
- 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.
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.
Risk factors
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 less 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.
What are the signs and symptoms of vulvar cancer?
Vulvar cancer may not cause noticeable early symptoms.
If you have one or more of the following symptoms, see your healthcare provider for an examination.
- Changes in the vulvar skin color (more red or white/pale than normal).
- Growths or lumps in the vulva that look like a wart or ulcer; or a rash or other sore that doesn’t heal.
- Itching or burning in the vulvar area that does not go away.
- Bleeding in the vulvar area not related to menstruation (periods).
- Tenderness in the vulvar area.
- Pelvic pain while having sex or peeing.
Complications
Complications from vulvar carcinoma are rare. If the disease progresses, complications include lymphedema, cellulitis, bone metastasis, and urethral obstruction.
Diagnosis and staging
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.
Treatment of Vulvar Cancer
Treatment options for vulvar cancer depend on the type, stage and location of your cancer, as well as your overall health and your preferences.
Surgery
Operations used to treat vulvar cancer include:
- Removing the cancer and a margin of healthy tissue (excision). This procedure, which may also be called a wide local excision or radical excision, involves cutting out the cancer and a small amount of normal tissue that surrounds it. Cutting out what doctors refer to as a margin of normal-looking tissue helps ensure that all of the cancerous cells have been removed.
- Removing part of the vulva or the entire vulva (vulvectomy). Surgery to remove part of the vulva (partial vulvectomy) or the entire vulva, including the underlying tissue (radical vulvectomy), may be an option for larger cancers. People with larger cancers may also consider treatment that combines radiation therapy and chemotherapy to shrink the tumor before surgery, which may allow for a less extensive operation.
- Removing a few nearby lymph nodes (sentinel node biopsy). To determine whether cancer has spread to the lymph nodes, the surgeon may use a procedure called sentinel node biopsy. This procedure identifies the lymph nodes most likely to contain cancer so they can be removed and analyzed. If cancer isn’t found in those first lymph nodes, it’s unlikely to be in any other lymph nodes.
- Removing many lymph nodes. If the cancer has spread to the lymph nodes, many lymph nodes may be removed to reduce the risk that cancer will spread to distant areas of the body.
Surgery carries a risk of complications, such as infection and problems with healing around the incision. Removing lymph nodes can cause fluid retention and leg swelling, a condition called lymphedema.
Radiation therapy
Radiation therapy uses high-powered energy beams, such as X-rays and protons, to kill cancer cells. Radiation therapy for vulvar cancer is usually administered by a machine that moves around your body and directs radiation to precise points on your skin (external beam radiation).
Radiation therapy is sometimes used to shrink large vulvar cancers in order to make it more likely that surgery will be successful. Radiation therapy is sometimes combined with chemotherapy, which can make cancer cells more vulnerable to the radiation.
If cancer cells are discovered in your lymph nodes, your doctor may recommend radiation to the area around your lymph nodes to kill any cancer cells that might remain after surgery. Radiation is sometimes combined with chemotherapy in these situations.
Chemotherapy
Chemotherapy is a drug treatment that uses chemicals to kill cancer cells. Chemotherapy drugs are typically administered through a vein in your arm or by mouth.
For those with advanced vulvar cancer that has spread to other areas of the body, chemotherapy may be an option.
Chemotherapy is sometimes combined with radiation therapy to shrink large vulvar cancers in order to make it more likely that surgery will be successful. Chemotherapy may also be combined with radiation to treat cancer that has spread to the lymph nodes.
Targeted drug therapy
Targeted drug treatments focus on specific abnormalities present within cancer cells. By blocking these abnormalities, targeted drug treatments can cause cancer cells to die.
Targeted therapy might be an option for treating advanced vulvar cancer.
Immunotherapy
Immunotherapy uses your immune system to fight cancer. Your body’s disease-fighting immune system may not attack your cancer because the cancer cells produce proteins that help them hide from the immune system cells. Immunotherapy works by interfering with that process.
Immunotherapy might be an option for treating advanced vulvar cancer.
Follow-up tests after treatment
After completing vulvar cancer treatment, your doctor may recommend periodic follow-up exams to look for a cancer recurrence. Even after successful treatment, vulvar cancer can return. Your doctor will determine the schedule of follow-up exams that’s right for you, but doctors generally recommend exams two to four times each year for the first two years after vulvar cancer treatment.
Vulvar Cancer Prevention
The cause of vulvar cancer is not known at this time. However, certain risk factors are thought to contribute to development of the disease. Suggestions for prevention include:
- Avoiding known risk factors when possible
- Delaying onset of sexual activity
- Using condoms
- Not smoking
- Having regular physical checkups
- Getting vaccinated against HPV
- Having routine Pap tests and pelvic exams
- Routinely checking entire body for irregular growth of moles and checking your vulva regularly for any signs of vulvar cancer
The HPV vaccine can prevent the strains of HPV responsible for most cervical, vaginal and vulvar cancers. HPV vaccines can only be used to prevent certain types of HPV. They cannot be used to treat an existing HPV infection. To be most effective, one of the vaccines should be given before a person becomes sexually active.