What Is Vulvar Cancer?
Vulvar cancer is an abnormal growth of malignant (cancerous) cells in the vulva. The vulva is defined as the external female genitalia and includes the labia majora (outer lips of labia), labia minora (inner lips), clitoris, mons pubis, vestibule, or entryway, of the vagina, and the perineum (area between vulva and anus).
About 80% of vulvar cancers involve the labia, mainly the labia majora (~50%). About 10% involve the clitoris, and another 10% involve the perineum. In about 5% of cases, the cancer is present at more than one site.
Vaginal cancer is more rare, and often treated similarly to vulvar cancer.
What are the different types of vulvar cancer?
The vulva is essentially epithelial skin; therefore the main tumor types that affect the vulva are skin-related cancers.
About 90% of vulvar cancers are squamous cell carcinomas, which typically develop at the edges of the labia majora/ minora or in the vagina. As with vaginal squamous cell carcinomas, vulvar squamous cell cancers are slow-growing and usually develop from “precancerous”, pre-invasive areas called vulvar intraepithelial neoplasia (VIN). There are two subtypes of squamous cell vulvar cancer. One is more common in younger persons and is caused by the human papillomavirus (HPV); the other occurs in older persons and is not associated with HPV infection, but is associated with chronic vulvar skin changes called vulvar dystrophy, including lichen sclerosus.
Melanoma is the second most common type of cancer found in the vulvar area and represents less than 5% of vulvar cancer cases. The most common skin cancer in sun-exposed areas is basal cell carcinoma, and as expected, this type rarely occurs on the vulva.
A sarcoma of the soft tissue can develop in the vulva and these account for 1-2% of vulvar cancers. Adenocarcinomas of the vulva are also rare but can develop from glands, most commonly the Bartholin’s glands located at the vaginal opening.
Am I at risk for vulvar cancer?
Vulvar cancer is a relatively rare cancer, representing about 6% of all gynecologic cancers, and only about .4% of all cancers.
Vulvar cancer most commonly occurs in postmenopausal persons. The peak age of diagnosis is between 70-79 years old. However, the rate of vulvar cancers diagnosed in younger persons has been increasing in the last decade, as a result of cancers caused by the Human Papilloma Virus (HPV) infection. These HPV-associated vulvar cancers are often seen in persons under 45 years of age. Experts agree that these two groups have different types of squamous cell vulvar cancer, which behave differently and respond differently to treatment.
In post-menopausal persons, vulvar cancer is often associated with long-term changes in the vulvar skin (vulvar dystrophy, squamous cell hyperplasia, or lichen sclerosus). This may be thickening or thinning of the vulvar skin or a white area that may be itchy or painful.
HPV Associated Vulvar Cancers
More than half of vulvar cancers are caused by HPV infection. HPV is a sexually transmitted disease that is very common in the population. Most college-aged persons have been exposed to HPV, though in most cases, the immune system inactivates or clears the virus from the body. There are over 100 different subtypes, or strains, of HPV and only certain subtypes are “oncogenic”, or able to cause cancer (these include: HPV 16,18, 33, 39, among others). Certain strains of HPV cause genital warts, though these strains are not oncogenic. Infection with HPV typically causes no symptoms, and may only be detected when a woman has an abnormal pap result or HPV testing that may be done along with the pap test. It is important to know that only a very small percentage of those who have a high-risk strain of HPV will develop cancer caused by the virus; so simply having HPV does not mean that you will get cancer.
HPV-associated vulvar cancers may appear in more than one location and may occur in conjunction with cervical, vaginal or perianal cancers, as these are also caused by HPV infection.
Being a current smoker is also considered a risk factor. Smoking is linked to an inability of the body’s immune system to clear an HPV infection; therefore, smokers are more likely to develop chronic HPV infections that may lead to cancer.
HIV infection or a history of other gynecologic cancer or melanoma can also increase the risk of developing vulvar cancer.
How can I prevent vulvar cancer?
It may be possible to lower your risk of developing vulvar cancer by avoiding HPV exposure, and by stopping or never starting to smoke. Pap smear/pelvic examination may identify early signs of vulvar cancer, so having this exam performed regularly is suggested (every 3-5 years depending on age and HPV status).
Additionally, research has shown that supplementation with active hexose-correlated compound, a mushroom extract, for at least 6 months has been associated with a 60% successful elimination of human papillomavirus (HPV) infections in those with positive PAP smears.
What are the symptoms of vulvar cancer?
The classic symptom is vulvar itching (pruritus) is reported in almost 90% of persons with vulvar cancer. There can also be associated pain, pain with intercourse, bleeding, vaginal discharge, and/or painful urination (dysuria). Precancerous lesions or early vulvar cancers may have mild or minimal symptoms. Preventive gynecologic exams can be helpful in detecting these early lesions.
Ultimately, many will develop a visible vulvar mass: the squamous cell subtype can look like elevated white, pink, or red bumps, while vulvar melanoma characteristically presents as a colored, ulcerated growth. There can be portions of the tumor that look sore and scaly, or cauliflower-like (similar to HPV-related warts).
How is vulvar cancer diagnosed?
First and foremost, a thorough gynecological examination should be performed using colposcopy and/or vulvoscopy, which uses a special magnifying instrument for better visualization. Any abnormal-appearing area(s) should be biopsied. Up to 50% of the time, cancer may be “multi-focal”; meaning that the cancer is in two separate places.
CT scan or MRI of the abdomen/pelvis may be done to look for disease spread to lymph nodes and/or distant organs in advanced cases, but is not necessary for early-stage disease.
How is vulvar cancer staged?
Vulvar cancer can spread by direct extension, meaning that it can grow into adjacent areas such as the vagina and anus. Even in early disease, spread to lymph nodes can occur. However, spread to other organs is rare until late in the course of the disease.
Once a diagnosis is confirmed, vulvar cancer is staged. Staging helps the healthcare team better understand the prognosis and decide which treatment options are best for each individual.
What are the treatments for vulvar cancer?
Surgery, radiation therapy, and chemotherapy are the typical treatment options. Depending on the cancer stage they may be used as a single treatment modality or in combination.
Recurrence of Vulvar Cancer
Local recurrence (near where the cancer was originally found) is the most common place where cancers can recur. These can often be treated with surgery (re-excision). If the disease has spread to other organs (metastatic or distant disease), it may be treated with chemotherapy. The most frequently used chemotherapy regimens are called “platinum-based”, meaning they consist of cisplatin or carboplatin, given alone or combined with another agent, such as vinorelbine, paclitaxel, erlotinib, gemcitabine, and pembrolizumab.
Side Effects of Vulvar Cancer & Cancer Treatment
Many of the side effects of surgery and radiation occur due to the close proximity of the bladder and rectum to the vulva. Due to this close proximity, these organs can be damaged during surgery or radiation. Side effects from the radiation can include irritation of the bowel and bladder resulting in diarrhea and increased frequency or urgency of bowel movements or urination. This typically resolves within a few weeks of finishing treatment, though it can become a long-term concern for some.
Radiation can cause scar tissue to form in the vagina and the tissue can become dry and less elastic. There may be some shrinking of the vagina and vaginal opening. Scarring of the vaginal tissue can result in “adhesions”, or areas where scar tissue forms, sealing the sides of the vagina together. This can make it difficult for a provider to perform vaginal exams and makes sexual intercourse difficult and uncomfortable. Your oncology team will teach you to use vaginal dilators to reduce the severity of this side effect. Rarely, a connection between the bladder or rectum and the vagina can form (also known as a fistula), which allows passage of stool or urine into the vagina.
Damage to the drainage (lymphatic) system in the area, by radiation or surgery to remove lymph nodes, can lead to a chronic swelling called lymphedema, which can occur at any time after treatment. Studies have found this condition to occur in anywhere between 14-48% of those who undergo lymph node dissection for vulvar cancer. Notify your healthcare provider if you develop any swelling in the legs or pelvis. A survivor with lymphedema who develops pain or redness in the leg(s), especially with fever, should be evaluated right away, as these signs may indicate infection and can be life-threatening if not treated promptly. Some may be good candidates for sentinel lymph node biopsy (SLNB), which appears to greatly reduce the risk of developing lymphedema. In SLNB, only the lymph nodes that the tumor area drains to are removed, limiting the damage done to the lymph system. SLNB is performed by gynecologic oncologists that have been trained in this procedure.
Follow-Up Care and Survivorship
After treatment for vulvar cancer, you will be followed closely by your healthcare team. In general, is it recommended that you have a pelvic exam every 3-6 months for the first 2 years after treatment, then every 6-12 months for years 3-5 after treatment, and then annually based on risk for recurrence. Other tests including cervical/vaginal cytology screenings, radiology, CT or PET scans, and lab tests may also be performed depending on symptoms or suspicions of recurrence.
More than half of those who undergo vulvectomy report sexual dysfunction and psychological issues. The extreme changes in a woman’s anatomy can result in painful intercourse, body image concerns, decreased desire, inability to orgasm, and difficulty with urination. Adequately preparing a woman for the expected changes, providing tools for coping with these changes, and discussing the emotions surrounding cancer and its treatment may help some. One should not hesitate to talk openly with their healthcare providers about their feelings and fears and consider professional counseling to help in healing emotionally, as well as physically.
Fear of recurrence, the financial impact of treatment, employment issues, and coping strategies are other common emotional and practical challenges experienced by vulvar cancer survivors. Your healthcare team can identify resources for support and management of these challenges faced during and after cancer.
Resources for Patients
Foundation for Women’s Cancers
Offers comprehensive information by cancer type that can help guide you through your diagnosis and treatment. Also offers the ‘Sisterhood of Survivorship’ to connect with others facing similar challenges.
Cancer Care
Provides education, resources and support both online and by phone.
Chemocare
Provides information and resources about chemotherapy drugs
Sharp Hospital Cancer Support Groups and Services
Sharp offers a comprehensive range of support services to help you and your loved ones manage your cancer diagnosis and treatment.
Scripps Cancer Support Groups and Services
Scripps MD Anderson offers an array of cancer support programs, services and resources to help you every step of the way.
References
All About Vulvar Cancer | OncoLink
(SGO 2019: Mushroom Extract Supplementation Is Linked to Over 60% Eradication