Vulvar Cancer: Symptoms, Causes & Treatment Guide

What Is Vulvar Cancer?

Vulvar cancer is a malignant tumor that develops in the vulva, the external female genital area including the labia, clitoris, and vaginal opening. It accounts for approximately 4% of all gynecologic cancers and primarily affects postmenopausal women, with the highest incidence in women over 70 years of age.

The vulva is the outer part of the female reproductive system and includes several structures: the labia majora (outer lips), labia minora (inner lips), clitoris, the opening of the vagina, and the opening of the urethra. Vulvar cancer most commonly develops on the labia, particularly the labia majora, but can occur anywhere on the vulva.

Understanding vulvar cancer begins with recognizing that it develops when cells in the vulvar tissue undergo genetic changes that cause them to grow uncontrollably. Unlike many cancers that develop internally, vulvar cancer often causes visible changes that can be detected during self-examination or routine gynecological visits, which makes early detection possible when women are aware of what to look for.

The condition was historically considered a disease primarily affecting elderly women, but in recent decades, there has been an increase in cases among younger women, largely attributed to the rising prevalence of human papillomavirus (HPV) infection. This shift has important implications for prevention strategies, including HPV vaccination.

Types of Vulvar Cancer

Several different types of cancer can develop in the vulva, each with distinct characteristics and behaviors:

Squamous cell carcinoma is by far the most common type, accounting for approximately 90% of all vulvar cancers. This type develops from the flat, thin cells (squamous cells) that line the surface of the vulva. Squamous cell carcinoma can be further divided into HPV-related and HPV-independent subtypes, which have different risk factors and may behave differently.

Melanoma of the vulva accounts for approximately 5-8% of vulvar cancers. Like melanoma elsewhere on the body, vulvar melanoma develops from the pigment-producing cells (melanocytes) and tends to be more aggressive than squamous cell carcinoma. It often appears as a dark-colored lesion but can sometimes lack pigmentation.

Adenocarcinoma develops from glandular tissue in the vulva, particularly the Bartholin's glands. This type is relatively rare and may require different treatment approaches than squamous cell carcinoma.

Basal cell carcinoma can occasionally develop on the vulva, similar to the basal cell carcinomas that commonly occur on sun-exposed skin. This type typically grows slowly and rarely spreads to other parts of the body.

Paget's disease of the vulva is an uncommon condition where cancer cells develop in the surface layer of the vulvar skin. It appears as a red, scaly patch and may be associated with an underlying adenocarcinoma.

Precancerous Conditions

Before vulvar cancer develops, there are often precancerous changes in the vulvar tissue that can be detected and treated. The main precancerous condition is called vulvar intraepithelial neoplasia (VIN), which refers to abnormal cells confined to the surface layer of the vulvar skin.

VIN is classified based on whether it is HPV-related (usual type VIN) or HPV-independent (differentiated VIN). Usual type VIN is more common in younger women and is associated with HPV infection, while differentiated VIN typically occurs in older women, often in the setting of chronic inflammatory conditions like lichen sclerosus. Differentiated VIN, although less common, has a higher risk of progressing to invasive cancer.

What Are the Symptoms of Vulvar Cancer?

The most common symptoms of vulvar cancer include persistent itching (pruritus), a visible lump or mass, changes in skin color or texture, pain or burning sensation, and bleeding or discharge unrelated to menstruation. An open sore or ulcer that doesn't heal is also a significant warning sign that requires medical evaluation.

Recognizing the symptoms of vulvar cancer is crucial for early detection and successful treatment. Many women delay seeking medical attention because the symptoms can be subtle, embarrassing to discuss, or mistaken for other common conditions. Understanding what to look for can help women identify potential problems early.

The presentation of vulvar cancer can vary considerably between individuals. Some women experience multiple symptoms simultaneously, while others may notice only a single change. The location, size, and type of the cancer all influence what symptoms develop. It's important to note that having these symptoms doesn't necessarily mean you have cancer, as many benign conditions can cause similar problems, but any persistent changes should be evaluated by a healthcare provider.

Common Symptoms

Persistent itching is the most frequently reported symptom, occurring in approximately 50-60% of women with vulvar cancer. This itching (medical term: pruritus) is often chronic, doesn't respond to typical treatments for yeast infections or other common causes, and may have been present for months or even years before cancer is diagnosed. The itching may be localized to one area or more widespread.

A lump, bump, or mass on the vulva is another common finding. This may feel like a raised area, a wart-like growth, or a thickening of the skin. The lump may be painful or painless and can vary in size from very small to quite large. Any new growth on the vulva that persists for more than a few weeks should be examined by a healthcare provider.

Skin changes are often visible and may include changes in color (white patches, reddened areas, or darkened skin), changes in texture (thickening, roughness, or scaling), or the development of raised or flat lesions. White patches (leukoplakia) are particularly common and may represent precancerous changes or early cancer.

Pain, burning, or tenderness in the vulvar area may occur, especially as the cancer grows. This discomfort may be constant or may worsen with activities such as urination, sexual intercourse, or sitting. Some women describe a burning sensation or rawness rather than frank pain.

Bleeding that is not associated with menstruation can occur if the tumor involves blood vessels or if the skin surface breaks down. This may appear as spotting on underwear, bleeding during or after intercourse, or bleeding when the area is touched or cleaned.

An open sore or ulcer that doesn't heal within a few weeks is a significant warning sign. These ulcers may bleed easily, produce discharge, or become crusted over. Any wound on the vulva that doesn't heal with standard care should be evaluated.

Advanced Symptoms

As vulvar cancer progresses, additional symptoms may develop. These advanced symptoms often indicate that the cancer has grown larger or spread to nearby structures:

• Swollen lymph nodes in the groin area (inguinal lymphadenopathy)
• Difficulty urinating or painful urination if the tumor involves the urethra
• Difficulty with bowel movements if the tumor is near the anus
• Pain in the pelvis, groin, or legs
• Weight loss and general fatigue (in advanced cases)

What Causes Vulvar Cancer?

Vulvar cancer develops through two main pathways: HPV-related cancer (associated with human papillomavirus infection, particularly types 16 and 18) typically affecting younger women, and HPV-independent cancer (often associated with chronic inflammatory conditions like lichen sclerosus) typically affecting older women. Both pathways involve genetic mutations that cause vulvar cells to grow uncontrollably.

Understanding the causes and risk factors for vulvar cancer helps explain why certain women are more susceptible and what steps might be taken for prevention. Unlike some cancers with a single dominant cause, vulvar cancer can develop through different pathways, each with distinct risk factors and characteristics.

At the cellular level, vulvar cancer develops when DNA mutations accumulate in vulvar cells, disrupting the normal controls on cell growth and division. These mutations can be triggered by various factors, and the specific pathway determines the type of cancer that develops and may influence treatment approaches and prognosis.

HPV-Related Vulvar Cancer

Human papillomavirus (HPV) infection is a major cause of vulvar cancer, responsible for approximately 40-70% of cases. HPV is a very common sexually transmitted infection, with most sexually active people being exposed at some point in their lives. While most HPV infections clear on their own without causing problems, persistent infection with high-risk HPV types (particularly HPV 16 and HPV 18) can lead to cellular changes that eventually progress to cancer.

HPV-related vulvar cancer typically occurs in younger women, often those under 60 years of age. It is associated with usual type vulvar intraepithelial neoplasia (VIN) as a precursor lesion. Women with HPV-related vulvar cancer often have a history of other HPV-related conditions, such as cervical abnormalities, anal intraepithelial neoplasia, or genital warts.

The good news is that HPV-related vulvar cancer is largely preventable through vaccination. The HPV vaccine is highly effective at preventing infection with the HPV types most commonly associated with vulvar cancer and other HPV-related cancers.

HPV-Independent Vulvar Cancer

Not all vulvar cancers are caused by HPV. HPV-independent vulvar cancer typically occurs in older women and is often associated with chronic inflammatory conditions of the vulva, particularly lichen sclerosus. This type of cancer develops through a different pathway involving p53 tumor suppressor gene mutations.

Lichen sclerosus is a chronic skin condition that causes white, patchy skin that may be thin and wrinkled. It primarily affects the genital area and can cause significant itching and discomfort. While most women with lichen sclerosus will not develop vulvar cancer, the condition does increase the risk, with approximately 4-5% of women with lichen sclerosus eventually developing vulvar cancer over their lifetime.

Other chronic inflammatory conditions of the vulva may also increase the risk of HPV-independent vulvar cancer. These conditions cause ongoing inflammation and tissue changes that can eventually lead to malignant transformation.

Risk Factors

Several factors increase the risk of developing vulvar cancer:

• Age: The risk increases significantly with age, with most cases occurring in women over 65
• HPV infection: Persistent infection with high-risk HPV types, particularly HPV 16
• Smoking: Tobacco use increases the risk of HPV-related vulvar cancer by impairing the immune system's ability to clear HPV infections
• Lichen sclerosus: Chronic vulvar skin condition associated with increased cancer risk
• Weakened immune system: HIV/AIDS, organ transplant recipients on immunosuppressive drugs, or other conditions that weaken immunity
• History of cervical cancer or precancer: Women who have had cervical abnormalities have a higher risk of vulvar cancer
• Vulvar intraepithelial neoplasia (VIN): Precancerous changes in the vulvar skin

How Is Vulvar Cancer Diagnosed?

Vulvar cancer is diagnosed through physical examination followed by biopsy of suspicious areas. The biopsy provides tissue for microscopic examination to confirm cancer and determine its type. Additional tests including imaging (CT, MRI, or PET scans) and sentinel lymph node biopsy may be performed to determine the stage of the cancer.

Accurate diagnosis of vulvar cancer involves several steps, from initial recognition of suspicious symptoms or findings to definitive tissue diagnosis and staging. The diagnostic process is designed to confirm whether cancer is present, identify the type and characteristics of the cancer, and determine whether it has spread beyond the vulva.

Early diagnosis is crucial for successful treatment of vulvar cancer. Unfortunately, diagnosis is often delayed because women may be embarrassed to discuss vulvar symptoms, may attribute symptoms to other causes, or may not have regular gynecological care. Healthcare providers play an important role in asking about vulvar symptoms during examinations and thoroughly evaluating any reported concerns.

Physical Examination

The diagnostic process typically begins with a thorough physical examination of the vulva and surrounding areas. The healthcare provider will visually inspect the entire vulva, looking for any abnormalities such as lumps, color changes, ulcers, or unusual textures. They will also palpate (feel) the area to detect any masses or thickening that might not be visible.

The examination also includes checking the inguinal (groin) lymph nodes, as vulvar cancer commonly spreads to these nodes. Enlarged or firm lymph nodes may indicate that the cancer has spread beyond the vulva.

If the initial examination reveals any suspicious findings, additional evaluation is typically performed. This may include colposcopy, which uses a magnifying instrument to examine the vulva more closely, often with the application of acetic acid (vinegar) or other solutions that help highlight abnormal areas.

Biopsy

A biopsy is essential for diagnosing vulvar cancer. This procedure involves removing a small sample of tissue from the suspicious area so it can be examined under a microscope by a pathologist. There are several types of biopsies:

Punch biopsy uses a circular cutting tool to remove a small, cylindrical sample of tissue. This is the most common type of biopsy for suspected vulvar cancer and can usually be performed in a clinic setting with local anesthesia.

Excisional biopsy removes the entire suspicious area along with a margin of surrounding normal tissue. This may be both diagnostic and therapeutic for small lesions.

Incisional biopsy removes only a portion of a larger lesion. This is typically done when the lesion is too large to remove completely as an initial procedure.

The pathologist examines the biopsy tissue to determine whether cancer is present and, if so, what type of cancer it is. They also assess other characteristics that help guide treatment, such as the grade of the cancer (how abnormal the cells look) and whether HPV is involved.

Staging Workup

Once vulvar cancer is confirmed, additional tests are performed to determine the stage of the cancer - how far it has spread. Staging is crucial for planning treatment and predicting prognosis. The staging workup may include:

Imaging studies such as CT (computed tomography) scan, MRI (magnetic resonance imaging), or PET (positron emission tomography) scan help visualize the extent of the tumor and check for spread to lymph nodes or distant organs.

Sentinel lymph node biopsy is a procedure to identify and remove the first lymph nodes that drain the area where the cancer is located. If these nodes are cancer-free, it's likely that the cancer hasn't spread to other lymph nodes, and more extensive lymph node surgery may be avoided.

Examination under anesthesia may be performed to thoroughly evaluate the extent of the tumor and its relationship to surrounding structures, particularly if surgery is being planned.

What Are the Stages of Vulvar Cancer?

Vulvar cancer is staged using the FIGO system, ranging from Stage I (small tumor confined to the vulva) to Stage IV (spread to distant organs). Stage I has an 86% five-year survival rate, while Stage IV has approximately 19%. Staging is based on tumor size, depth of invasion, lymph node involvement, and distant spread.

Staging describes the extent of cancer in the body and is essential for determining the best treatment approach and understanding prognosis. Vulvar cancer is staged using the International Federation of Gynecology and Obstetrics (FIGO) staging system, which was updated in 2009.

The staging system considers several factors: the size of the tumor, how deeply it has invaded into surrounding tissues, whether it has spread to nearby lymph nodes, and whether it has spread to distant organs. Understanding your cancer's stage can help you and your healthcare team make informed decisions about treatment.

Stage I - Localized Cancer

Stage I cancer is confined to the vulva or perineum (the area between the vulva and anus), with no spread to lymph nodes. It is subdivided based on size and depth of invasion:

• Stage IA: Tumor is 2 cm or smaller and has invaded no more than 1 mm into the tissue (stromal invasion). This early stage has an excellent prognosis.
• Stage IB: Tumor is larger than 2 cm, OR has stromal invasion greater than 1 mm. Still confined to the vulva/perineum without lymph node spread.

Stage II - Larger Local Tumor

Stage II means the tumor has spread to nearby structures such as the lower part of the urethra, lower part of the vagina, or the anus, but has not spread to lymph nodes or distant sites. The tumor can be any size.

Stage III - Lymph Node Involvement

Stage III indicates that the cancer has spread to regional lymph nodes in the groin (inguinofemoral lymph nodes). It is subdivided based on the extent of lymph node involvement:

• Stage IIIA: Cancer has spread to 1-2 lymph nodes, with each involved node having metastases smaller than 5 mm, OR one lymph node with metastasis 5 mm or larger.
• Stage IIIB: Cancer has spread to 3 or more lymph nodes with metastases smaller than 5 mm each, OR 2 or more nodes with metastases 5 mm or larger.
• Stage IIIC: Cancer has spread to lymph nodes with extracapsular spread (cancer breaking through the outer covering of the lymph node).

Stage IV - Advanced Cancer

Stage IV is the most advanced stage and is divided into:

• Stage IVA: The cancer has spread to the upper part of the urethra, upper vagina, bladder, rectum, or pelvic bone, OR has fixed (immovable) or ulcerated inguinofemoral lymph node metastases.
• Stage IVB: The cancer has spread to distant parts of the body, such as the lungs or liver.

How Is Vulvar Cancer Treated?

The primary treatment for vulvar cancer is surgery, which may range from wide local excision for small tumors to more extensive vulvectomy for larger cancers. Treatment often includes sentinel lymph node biopsy or lymph node dissection. Radiation therapy and chemotherapy may be used in combination with surgery or for advanced cases.

Treatment of vulvar cancer has evolved significantly over the past several decades, moving toward more conservative approaches that aim to preserve as much normal tissue as possible while still effectively treating the cancer. The specific treatment plan depends on the stage of the cancer, its exact location, the patient's overall health, and her preferences.

A multidisciplinary team typically manages vulvar cancer treatment, including gynecologic oncologists, radiation oncologists, medical oncologists, pathologists, and other specialists. This team approach ensures that all treatment options are considered and that care is coordinated effectively.

Surgery

Surgery is the cornerstone of treatment for most vulvar cancers. The type of surgery depends on the size, location, and stage of the cancer:

Wide local excision removes the tumor along with a margin of healthy tissue surrounding it. This approach is used for smaller tumors and aims to preserve as much of the vulva as possible while ensuring all cancer is removed. The margin of normal tissue removed is typically at least 1-2 cm.

Radical local excision is a more extensive procedure that removes the tumor with wider margins and deeper tissue. This may be necessary for larger or deeper tumors.

Vulvectomy involves removal of part or all of the vulva. Partial (simple) vulvectomy removes only the affected portion, while radical vulvectomy removes the entire vulva including deeper tissues. Modern surgical approaches try to avoid extensive vulvectomy when possible to preserve sexual function and quality of life.

Sentinel lymph node biopsy is now standard for many vulvar cancers. This technique identifies and removes only the first lymph nodes that drain the tumor area. If these "sentinel" nodes are cancer-free, more extensive lymph node removal may be avoided, reducing complications like lymphedema.

Inguinofemoral lymph node dissection involves removing the lymph nodes in the groin area. This may be performed if sentinel lymph node biopsy is not possible or if sentinel nodes contain cancer.

Radiation Therapy

Radiation therapy uses high-energy beams to kill cancer cells. It may be used in several situations:

Adjuvant radiation is given after surgery to reduce the risk of recurrence, particularly if the cancer has spread to lymph nodes or if surgical margins are close or positive.

Neoadjuvant radiation (often combined with chemotherapy) may be given before surgery to shrink large tumors, making them easier to remove with less extensive surgery.

Primary radiation therapy may be the main treatment for women who cannot have surgery or for tumors in locations where surgery would significantly impact function.

Modern radiation techniques, such as intensity-modulated radiation therapy (IMRT), can target the tumor more precisely while minimizing damage to surrounding healthy tissues.

Chemotherapy

Chemotherapy uses drugs to kill cancer cells throughout the body. In vulvar cancer, chemotherapy is most commonly used:

In combination with radiation (chemoradiation) to enhance the effectiveness of radiation therapy. Cisplatin and 5-fluorouracil are commonly used agents.

For advanced or recurrent cancer that has spread beyond the vulva and cannot be effectively treated with surgery or radiation alone.

Immunotherapy

Immunotherapy is an emerging treatment option for vulvar cancer, particularly for cancers that are HPV-related or have specific molecular characteristics. Checkpoint inhibitors, which help the immune system recognize and attack cancer cells, have shown promise in some clinical trials for advanced vulvar cancer.

What Is the Prognosis for Vulvar Cancer?

The prognosis for vulvar cancer depends primarily on the stage at diagnosis. Early-stage vulvar cancer (Stage I) has an excellent prognosis with approximately 86% of patients surviving five years or more. Even with lymph node involvement (Stage III), approximately 53% of patients survive five years. Factors affecting prognosis include tumor size, depth of invasion, lymph node status, and tumor grade.

Prognosis refers to the likely course and outcome of a disease. For vulvar cancer, the most important factor determining prognosis is the stage of the cancer at the time of diagnosis. This underscores the importance of early detection and prompt treatment.

Survival statistics are based on the outcomes of many patients with vulvar cancer and represent averages. Individual outcomes can vary significantly, and many patients live well beyond what statistics might suggest. Advances in treatment continue to improve outcomes for women with vulvar cancer.

Factors Affecting Prognosis

Several factors influence the prognosis for an individual with vulvar cancer:

Stage at diagnosis is the most important prognostic factor. Localized cancers that haven't spread to lymph nodes have much better outcomes than those with lymph node involvement or distant spread.

Lymph node status is particularly important. The presence of cancer in lymph nodes significantly affects prognosis. The number of involved nodes, the size of metastases within nodes, and whether cancer has spread outside the lymph node (extracapsular extension) all influence outcomes.

Tumor characteristics including size, depth of invasion, grade (how abnormal the cells appear), and type of cancer also affect prognosis. Larger tumors, deeper invasion, and higher-grade cancers generally have a worse prognosis.

Surgical margins - whether all cancer was removed with a margin of normal tissue - is important for predicting recurrence risk. Positive margins (cancer at the edge of the removed tissue) are associated with higher recurrence rates.

Age and overall health influence both treatment options and outcomes. Younger, healthier patients often tolerate more aggressive treatment and may have better outcomes.

Recurrence

Vulvar cancer can recur (come back) after treatment. Recurrence most commonly occurs at or near the original tumor site (local recurrence) or in the groin lymph nodes. Distant recurrence (spread to organs like the lungs or liver) is less common but more serious.

Regular follow-up care after treatment is essential to detect any recurrence early when it may still be treatable. Follow-up typically includes regular examinations of the vulva and groin area, and imaging studies may be performed if there are concerning symptoms.

Can Vulvar Cancer Be Prevented?

While not all vulvar cancers can be prevented, several strategies can reduce risk. HPV vaccination significantly reduces the risk of HPV-related vulvar cancer. Other preventive measures include avoiding smoking, practicing safe sex, getting regular gynecological examinations, and promptly treating vulvar conditions like lichen sclerosus.

Prevention of vulvar cancer involves addressing modifiable risk factors and detecting precancerous changes before they progress to cancer. While some risk factors like age cannot be changed, others can be addressed through lifestyle modifications and medical interventions.

HPV Vaccination

HPV vaccination is one of the most effective ways to prevent HPV-related vulvar cancer. The HPV vaccine protects against the HPV types that cause most HPV-related cancers, including vulvar cancer. Vaccination is most effective when given before exposure to HPV, which is why it's recommended for adolescents, but it can provide benefit at older ages as well.

The vaccine is recommended for all individuals ages 9-26, and may be given up to age 45 for some adults who haven't been previously vaccinated. Even in women who have been sexually active, the vaccine can protect against HPV types they haven't yet been exposed to.

Lifestyle Modifications

Not smoking is important because smoking increases the risk of HPV-related vulvar cancer and interferes with the body's ability to clear HPV infections. Quitting smoking at any age can reduce this risk.

Practicing safe sex can reduce exposure to HPV. While condoms don't completely prevent HPV transmission (since HPV can infect areas not covered by condoms), they do provide some protection.

Limiting sexual partners can also reduce HPV exposure, although many people are infected with HPV through even one sexual partner.

Regular Screening and Self-Examination

Unlike cervical cancer, there is no routine screening test for vulvar cancer. However, regular gynecological examinations allow healthcare providers to identify suspicious changes early. Women should attend recommended check-ups and report any vulvar symptoms promptly.

Vulvar self-examination can help women become familiar with their normal anatomy and notice changes early. Using a hand mirror and good lighting, women can periodically examine their vulva for any changes in appearance or feel. Any new lumps, skin changes, sores, or unusual symptoms should be reported to a healthcare provider.

Managing Precancerous Conditions

Prompt treatment of vulvar intraepithelial neoplasia (VIN) can prevent progression to invasive cancer. VIN can be treated with surgical excision, laser therapy, or topical medications, depending on its extent and characteristics.

Women with lichen sclerosus should have regular monitoring, as this condition increases vulvar cancer risk. While treatment of lichen sclerosus (typically with potent topical corticosteroids) improves symptoms and may reduce cancer risk, ongoing surveillance is important.

When Should You See a Doctor?

See a doctor promptly if you notice any persistent vulvar symptoms including itching that doesn't respond to treatment, a new lump or bump, changes in skin color or texture, an open sore that doesn't heal, unexplained bleeding, or persistent pain or burning. Early evaluation of these symptoms can lead to earlier diagnosis and better outcomes.

Knowing when to seek medical attention for vulvar symptoms is crucial for early detection of vulvar cancer or its precursors. Many women hesitate to seek care for vulvar concerns due to embarrassment or dismissal of symptoms as minor, but prompt evaluation is important.

Remember that many vulvar conditions are not cancer, but early evaluation allows for proper diagnosis and treatment. If you have risk factors for vulvar cancer, such as a history of HPV infection, lichen sclerosus, or previous vulvar abnormalities, be especially vigilant about reporting any changes to your healthcare provider. In a medical emergency, call your local emergency number immediately.