This page was reviewed under our medical and editorial policy by
Maurie Markman, MD, President, Medicine & Science
This page was updated on June 7, 2022.
Vulvar cancer begins in the tissues of the vulva, the external portion of the female genitalia. It’s typically a slow-growing cancer.
The disease most often begins in the labia majora (the outer vaginal lips), though it may also affect the labia minora (inner vaginal lips), clitoris or vaginal glands. Women with this cancer are often misdiagnosed and may be treated for a rash or skin irritation for months or years before being diagnosed with cancer.
Vulvar cancer is rare, accounting for less than 1 percent of all cancers in women and about 6 percent of cancers of the female reproductive organs, according to the American Cancer Society. More than half of vulvar cancers are diagnosed in women over the age of 70.
Nearly 60 percent of women are diagnosed when the disease is localized (has not spread). The five-year survival for localized vulvar cancer is 86.3 percent.
Vulvar cancer has two main types:
Squamous cell carcinomas have cancer cells in the outer layer of the skin. This type accounts for more than 80 percent of vulvar cancers.
Adenocarcinoma is much rarer than squamous cell carcinoma of the vulva. This disease begins in the cells of the Bartholin glands, which are located on each side of the opening of the vagina.
Although the exact cause of vulvar cancer is not known, some squamous cell carcinomas of the vulva have been associated with the human papilloma virus (HPV). HPV-related squamous cell carcinoma of the vulva has distinctive features. These may include a skin disease known as intraepithelial neoplasia, characterized by a thickening or roughness of the vulvar skin and/or parts of the vulva appearing whiter or redder than the skin surrounding it. Women with this type of cancer tend to be in their 30s and 40s and are often smokers.
Other squamous cell carcinomas of the vulva do not appear to be related to HPV. A DNA mutation in the TP53 tumor suppressor gene is often detected in older women diagnosed with this type of vulvar cancer.
The most common symptoms of vulvar cancer are itching and a palpable mass (a lump or bump). The lump may be a different color—lighter or darker—than the skin surrounding it, and it may also feel rough or thick, similar to a wart. It may or may not be painful. Other symptoms include bleeding or discharge from the vulva, as well as swelling.
A gynecologic oncologist is a specialist trained in diagnosing and treating cancers of the female reproductive system, including vulvar cancer. These highly specialized physicians complete years of post-medical school training that includes instruction on surgical procedures, chemotherapy, radiation, immunotherapy and targeted therapy for gynecological cancers.
As with any cancer, the stage of the patient’s cancer, as well as her age, lifestyle and general health are taken into account when considering treatment options.
Surgery is the primary treatment for most vulvar cancers. It’s important for the patient and doctor to discuss the pros and cons, including sexual side effects, of possible surgical procedures. Common procedures include those listed below.
Excision: In this procedure, the surgeon removes the cancer and usually about a half-inch of healthy skin, known as a margin, from each side of the vulva. A thin layer of fat beneath the cancer is also typically excised, or cut out, during the procedure.
Pelvic exenteration: This is a complex operation during which the surgeon removes the vulva and pelvic lymph nodes. If the cancer has metastasized to nearby organs or other body parts, such as the lower colon, rectum, bladder, uterus, cervix and/or vagina, they may also be removed.
Vulvectomy: In this procedure, the surgeon removes all or part of the vulva. Various types of vulvectomies may be performed. These may include minimal skin removal—known as a skinning vulvectomy—or simple procedures, such as surgery to remove the entire vulva and tissue just beneath the skin, or a partial or modified radical vulvectomy (removing part of the vulva as well as deep tissue). In rare cases, a complete radical vulvectomy that removes the entire vulva, deep tissues and clitoris may be necessary.
Lymph node surgery: Two types of lymph node surgeries are associated with vulvar cancer: sentinel lymph node biopsy and an inguinal lymph node dissection. A sentinel lymph node biopsy may be performed to identify and test the nodes where the cancer cells are most likely to have spread first. If these lymph nodes do not have cancer cells in them, no additional lymph nodes need to be removed. If cancer is found in the sentinel lymph nodes, the surgeon will remove surrounding nodes. An inguinal lymph node dissection is performed to remove lymph nodes on one or both sides of the groin.
Chemotherapy, radiation therapy or the combination of the two, known as chemoradiation, may also be used to treat vulvar cancer.
Chemotherapy drugs attack healthy cells along with cancerous ones, which may result in side effects. The type and amount of medication, as well as the length of treatment may determine which side effects impact the patient's quality of life, and how severely. Common side effects of chemotherapy for vulvar cancer may include:
Side effects from radiation therapy may include:
The extent of sexual side effects, if any, and how long they last may vary greatly depending on the type of surgery the patient had. Following any surgery, patients typically experience bruising and swelling that eventually heals.
After a vulvectomy, the vagina will look different. A radical or modified radical vulvectomy will change the vagina’s appearance more than a partial vulvectomy, particularly if the labia minora and majora (inner and outer lips) have been removed. Reconstructive surgery, which is an option for some women, may change the sensation in the vulva. Scarring may also lead to numbness and/or pain during sex. Persistent swelling in the legs and/or genital region—a condition called lymphedema—sometimes affects women who have had lymph nodes in the groin removed, often making sex painful. The ability to have an orgasm may be impacted, depending on how much of the vulva and/or clitoris and lower vagina are removed.
It’s important for patients to communicate with their partner and their doctor about physical, sexual and psychological discomfort, because a number of therapies, techniques and procedures are available to reduce these side effects.
During the vulvar cancer treatment regimen, it is important to follow the care team’s instructions and know, in advance, the potential side effects and strategies for managing them.
If the patient's treatment involves chemotherapy, radiation or chemoradiation, it’s likely she will experience, at least temporarily, some side effects. Prior to beginning treatment, talk to the care team about supportive care strategies like dietary supplements for fatigue and physical therapy for neuropathy (numbness, pain and/or tingling caused by nerve damage).
Every patient’s recovery experience and recovery time is unique and may vary depending on the extent of her surgery, as well as her age and general health prior to surgery.
Post-surgical pain or discomfort should be expected with any surgical procedure. Intravenous pain medication or an injection to numb the pain or block certain nerves (known as a nerve block) may be given immediately following surgery. If the patient is still experiencing pain after the first couple of days, the care team may prescribe prescription medications during recovery at home.
Constipation is the most common side effect from prescription-strength pain medications. Other common side effects include nausea, dizziness, drowsiness and depression. Opioids, a narcotic commonly given following major surgery, are also associated with a high risk of addiction. If used over an extended period of time, patients taking opioids may require higher and higher doses to achieve the same level of pain relief, increasing the risk of addiction. Other pain management strategies are available to control pain just as well, and they typically cause fewer side effects. Speak to the care team about options for managing pain before starting treatment.
Vulvar cancer surgery is associated with a high risk of complications, namely infection. Caring for the surgical site is imperative to reducing the infection risk. While the patient is still in the hospital, the care team will wash the area and apply the necessary medication to the site to help prevent infection. The care team will instruct the patient on how to care for the surgery wound at home. Once the patient leaves the hospital, it’s critical to her recovery to follow the instructions. Experts recommend wearing loose clothing to help the wound “breathe.” Contact the care team right away if swelling or redness, wound discharge or an unusual odor occurs.
Patients may feel overwhelmed after receiving a vulvar cancer diagnosis. It’s a good idea to carry a notebook and jot down questions as they arise, so they're handy at each appointment. The patient may have questions about treatment options or various surgical procedures or chemotherapy drugs. The more the patient knows about her disease, the better equipped she will be to make informed decisions that feel right.
Some questions to consider asking the care team include those listed below.
Determining the extent of cancer is called staging. The stage of the cancer indicates whether it exists in only one area (localized) or it has spread from the original site to elsewhere in the body. Stage 0 indicates an early-stage cancer restricted to the surface of the vulva. It may also be called "carcinoma in situ." Stage 4, the most advanced stage, indicates the tumor has spread throughout the body.
When cancer has spread from its original site, it’s known as metastasis. This is different from cancer that has returned to the same site, which is referred to as recurrent cancer. Vulvar cancer primarily spreads by invading surrounding tissue. The cancer cells may also travel to the lymph nodes. Treating metastatic vulvar cancer commonly includes a combination of surgery, radiation and chemotherapy. If surgery is not an option, the care team may recommend just chemotherapy and radiation. Before starting treatment, it’s a good idea to talk with the care team about potential side effects and ways to manage them.
The care team may use a variety of tests to determine the stage of the patient's cancer. Imaging tests commonly used to diagnose and stage vulvar cancer include chest X-ray, CT scan (a combination of X-rays that produce cross-sectional images of a specific area), MRI, PET scan (images taken using a radioactive drug to highlight disease that may not appear on other imaging tests), proctosigmoidoscopy and cystoscopy (to see if the cancer has spread to the rectum, colon or bladder) and a sentinel lymph node biopsy (a biopsy of the first lymph node(s) to which the cancer cells have most likely spread).
Cancer is a serious disease. Getting an accurate diagnosis and seeing an oncologist experienced in vulvar cancer are critical to the decision-making process. Every patient has a right to a second opinion. Getting confirmation of the diagnosis and/or treatment plan may provide peace of mind, or it may lead the patient in a different direction. A gynecologic oncologist treats cancers of the female reproductive tract, including vulvar cancer. Ask the care team about his or her experience treating the patient's cancer type, including her particular stage of cancer, as well as whether he or she is board-certified in any specialty. Find out which hospital(s) the doctor is affiliated with and whether the facility offers clinical trials. It’s important to feel comfortable with the care team before starting treatment.
Knowledge is empowering. In order to ask questions pertinent to each patient's particular type and stage of vulvar cancer, it’s important to learn as much as possible about the disease. Ask the care team to explain the patient's type and stage of cancer and which cells are involved. Also ask for recommendations for educational material about vulvar cancer and online forums or support groups that may help the patient connect with other women who have been through similar experiences.
Next topic: What are the facts about vulvar cancer?