Vulvar Cancer
Vulvar cancer is a rare form of cancer that originates in the tissues of the vulva, which is the collective term for the external sex organs of women and individuals assigned female at birth. The vulva encompasses various components, including:
- Opening of your vagina: This muscular canal serves functions related to sex, childbirth, and menstruation.
- Labia minora (inner lips): These are folds of tissue that surround the vaginal opening and extend above the clitoris.
- Labia majora (outer lips): The fleshy area that encircles the inner lips.
- The outer part of your clitoris: This is a sexually sensitive area situated above the vaginal opening.
- Mons pubis: The rounded region in front of your pubic bones, which becomes covered with hair during puberty.
- Opening of the urethra: This tube allows the elimination of urine.
- Perineum: The patch of skin located between the vagina and anus.
Vulvar cancer typically progresses slowly over several years, often beginning with the development of precancerous lesions in the outermost layer of the skin, known as vulvar intraepithelial neoplasia (VIN).
Types of Vulvar Cancer
Healthcare professionals classify vulvar cancer based on the specific type of cells in which the cancer originates. The most common types include:
- Vulvar squamous cell carcinoma: Approximately 90% of vulvar cancers fall into this category, developing in the skin’s surface cells.
- Vulvar melanoma: Around 5% of vulvar cancers are melanomas, which are characterized by rapid growth and a higher risk of spreading to other parts of the body.
Less common types of vulvar cancer encompass:
- Basal cell carcinoma
- Bartholin gland adenocarcinoma
- Paget disease of the vulva
- Sarcoma
- Verrucous carcinoma
How Common is Vulvar Cancer?
Vulvar cancer is considered rare, with healthcare providers diagnosing fewer than 6,500 new cases annually in the United States. The majority of diagnoses are in individuals over the age of 50, and the average age at diagnosis is 68, with over 80% of cases occurring in those over age 50.
Symptoms and Causes
What are the Symptoms of Vulvar Cancer?
Vulvar cancer’s initial signs often manifest as changes to the skin on the outer lips (labia majora) or inner lips (labia minora). These symptoms may include:
- Changes in skin color, such as darker or lighter patches, or areas of white skin.
- Thickened or rough skin patches.
- Growths like lumps, wart-like bumps, or ulcers that do not heal.
- Persistent itching or burning that does not improve.
- Unexplained bleeding unrelated to menstruation.
- Tenderness and pain, particularly during sexual intercourse or urination.
If you experience one or more of these symptoms, it is crucial to consult your healthcare provider, even though vulvar cancer symptoms tend not to appear in the early stages. Many of these symptoms may also be associated with noncancerous conditions, so your provider can help determine the cause.
What Causes Vulvar Cancer?
Vulvar cancer arises when cells in the vulva begin to multiply uncontrollably. The most common type, vulvar squamous cell carcinoma, is associated with two primary conditions:
- Human papillomavirus (HPV) infection: HPV is a prevalent sexually transmitted infection that can increase the risk of certain cancers, including vulvar cancer.
- Lichen sclerosus: This is a lifelong skin condition that causes inflammation, skin changes, and itching in the vulva.
Risk Factors
Several risk factors increase the likelihood of developing vulvar cancer, including:
- Age: The risk of vulvar cancer rises with age.
- Exposure to HPV: Certain strains of HPV can lead to cell changes that may eventually result in vulvar cancer.
- Skin conditions involving the vulva: Growths associated with lichen sclerosus can progress to vulvar cancer.
- Vulvar intraepithelial neoplasia (VIN): This precancerous condition can develop into vulvar cancer if left untreated.
- Human immunodeficiency virus (HIV) infection: Weakened immunity due to conditions like HIV can make it harder for the body to combat cancer.
- Smoking: Smoking is a risk factor for various cancer types, including vulvar cancer.
Diagnosis and Tests
How is Vulvar Cancer Diagnosed?
Diagnosing vulvar cancer involves a comprehensive evaluation of your medical history, potential risk factors, and symptoms. This diagnostic process typically includes several tests:
- Pelvic exam: Your healthcare provider will visually inspect your vulva for any unusual skin changes. They may also use gloved, lubricated fingers to feel for lumps or signs of cancer by examining the vagina and rectum. In some cases, a speculum may be used to widen the vaginal opening for further examination.
- Pap smear: During a pelvic exam, your provider may collect cell samples for testing for cancerous changes. They may also perform an HPV test on the sample to check for infection.
- Colposcopy: Your provider may use a colposcope, a magnifying instrument equipped with a light, to examine the vulva, vagina, and cervix in detail. This procedure can highlight abnormal cells, making them easier to identify.
- Biopsy: To definitively confirm the presence of vulvar cancer, your provider may remove a sample of abnormal tissue for testing. A biopsy is the most reliable method to determine whether cancer is present.
Tests to Determine Cancer Spread
In the event of a cancer diagnosis, your healthcare provider will conduct additional tests to assess whether the cancer has extended beyond the vulva. Vulvar cancer, if left untreated, can spread to the vagina, nearby organs, lymph nodes in the pelvis, and eventually into the bloodstream. The diagnosis of metastatic cancer (cancer that has spread) is more challenging to treat. These tests may encompass:
- Scope exams: A cystoscopy may be used to check for cancer spread in the urethra or bladder. Similarly, a proctoscopy can examine the rectum or anus for cancer cells.
- Imaging tests: X-rays, CT scans, MRI scans, and PET scans are imaging techniques that can reveal whether the cancer has disseminated from the vulva to other tissues.
- Sentinel node biopsy: This procedure involves the removal and examination of the lymph node closest to the tumor, known as the sentinel node, to check for cancer cells. With vulvar cancer, tumors typically drain into sentinel lymph nodes in the groin.
What are the Stages of Vulvar Cancer?
The staging of vulvar cancer is essential in determining the extent of cancer spread, which, in turn, guides treatment decisions. There are four primary stages:
- Stage I: Early-stage vulvar cancer is confined to the vulva or perineum (the region between the rectum and vagina). Stage I is further categorized into Stages IA or IB, based on tumor size and how deeply it has invaded nearby tissue.
- Stage II: At this stage, the tumor, regardless of its size, has extended into the lower part of the urethra, lower part of the vagina, or anus.
- Stage III: Cancer has spread to one or more nearby lymph nodes, and this stage includes subcategories IIIA, IIIB, and IIIC, depending on the number and size of the involved lymph nodes.
- Stage IV: Cancer has disseminated into the upper part of the urethra, vagina, or other distant body parts. Stage IV is further divided into stages IVA and IVB, depending on whether the spread is localized near the vulva or more distant.
Consult your healthcare provider for a detailed explanation of what your specific cancer stage means for your treatment.
Management and Treatment
How is Vulvar Cancer Treated?
The treatment approach for vulvar cancer is individualized, taking into account factors such as your overall health, cancer stage, and whether the diagnosis is recent or recurrent. Your healthcare provider will explain the most suitable treatment plan based on your specific situation.
- Surgery: Surgery is the most commonly employed treatment for vulvar cancer, with the goal of removing all cancer while preserving sexual function. Surgical options include:
- Laser surgery: This technique employs a laser beam for bloodless tissue incisions or the removal of cancerous surface lesions.
- Local excision: It involves the removal of the cancer and a variable amount of surrounding normal tissue. In some cases, nearby lymph nodes may also be removed for testing or if cancer is suspected.
- Vulvectomy: This procedure entails the partial or complete removal of the vulva, potentially followed by skin grafts to replace excised skin.
- Pelvic exenteration: A more extensive surgery that removes the lower colon, rectum, bladder, cervix, vagina, ovaries, and adjacent lymph nodes. This necessitates the creation of openings to facilitate urine and stool elimination.
- Radiation Therapy: Radiation therapy employs X-rays or other high-energy sources to eliminate cancer cells. External beam radiation therapy (EBRT) is the most common method of delivering radiation for vulvar cancer treatment. In some cases, radiation is combined with chemotherapy (chemoradiation) and may be administered before surgery to shrink a tumor or after surgery to eradicate any remaining cancer cells.
- Chemotherapy: Chemotherapy uses drugs to target cancer cells throughout the body. Administration may involve injections, oral medications, or topical lotions applied directly to the vulvar area. The choice of chemotherapy drugs can vary, but commonly used ones for vulvar cancer include cisplatin (Platinol®, Platinol -AQ®) and fluorouracil (Carac®).
- Immunotherapy: Immunotherapy enhances the body’s immune system’s ability to identify and combat cancer cells more effectively. Imiquimod cream (Aldara®, Zyclara®) is a common immunotherapy medication used in the treatment of vulvar cancer.
Follow-Up After Vulvar Cancer Treatment
After treatment, your healthcare provider will conduct regular follow-up tests to monitor your condition and ensure cancer has not recurred. These tests often involve the same procedures used for diagnosis and staging of vulvar cancer.
Prevention
Can Vulvar Cancer be Prevented?
The most effective method of reducing your risk of vulvar cancer is to receive the HPV vaccine to prevent infections. In the United States, adults up to age 45 may receive the Gardasil 9® vaccine, depending on their risk of HPV exposure. Cervarix® and Gardasil® are HPV vaccines available in other countries. Regular checkups, including an annual gynecological examination, are crucial for maintaining good vulvar health.
Outlook / Prognosis
Is Vulvar Cancer Serious?
Vulvar cancer can be a serious and life-threatening condition if left untreated. When cancer has spread to lymph nodes or other body parts, it becomes more challenging to treat compared to early-stage diagnoses.
Survival Rate for Vulvar Cancer
The relative five-year survival rate for people with vulvar cancer is approximately 70%. However, survival rates are notably higher when individuals are diagnosed and treated at an early stage. For instance, the five-year survival rate for localized cancer (confined to the vulva) is approximately 86%, while it drops to approximately 30% when cancer has spread.
It’s important to note that your prognosis depends on various factors unique to your diagnosis, including your overall health and your response to treatment. Your healthcare provider can provide more specific information about your likely outcomes.
How Quickly Does Vulvar Cancer Progress?
The majority of vulvar cancer types progress slowly over several years. Less common types, such as melanomas, tend to grow and spread more rapidly.
Living With
How Do I Take Care of Myself?
Many individuals with vulvar cancer may experience self-consciousness due to visible changes in their vulva. However, having vulvar cancer does not mean you have to give up on physical intimacy. Don’t hesitate to discuss the potential impact of your diagnosis on your sex life with your healthcare provider. They can offer resources and support to address both your physical and emotional needs as you navigate your diagnosis and treatment.
Additional Common Questions
Where Does Vulvar Cancer Usually Start?
Vulvar cancer typically originates on the surface of the skin surrounding the vagina, particularly on the outer lips (labia majora) or inner lips (labia minora). Less commonly, it may develop in other parts of the vulva, including the Bartholin gland and clitoris.
What Is the First Stage of Vulvar Cancer?
The first stage of vulvar cancer is categorized as Stage I. In this stage, cancer is confined to the vulva or perineum. Stage IA refers to tumors that are two centimeters or smaller in size and have not spread more than one millimeter into nearby tissue. Stage IB involves larger tumors that may have invaded deeper into surrounding tissue.