Vulvar Cancer: Symptoms, Causes & Treatment

Overview

Vulvar cancer is a relatively rare type of cancer that develops in the vulva, the external female genital area. The vulva includes the inner and outer labia, the clitoris, the vaginal opening, and the surrounding skin and tissue. This cancer most commonly affects the labia majora (outer lips) and labia minora (inner lips), though it can develop in any part of the vulvar region.

Accounting for approximately 4% of all gynecologic cancers and less than 1% of all female cancers, vulvar cancer affects about 6,000 women in the United States each year. The disease primarily occurs in postmenopausal women, with the average age at diagnosis being 68 years, although it can occur at any age, including in younger women.

There are several types of vulvar cancer, with squamous cell carcinoma being the most common, representing about 90% of cases. Other less common types include melanoma, basal cell carcinoma, adenocarcinoma, and sarcoma. The prognosis for vulvar cancer is generally favorable when detected early, with five-year survival rates exceeding 70% for localized disease.

Key Facts About Vulvar Cancer

Represents 4% of gynecologic cancers
Most common in women over 65 years
90% are squamous cell carcinomas
Early detection significantly improves outcomes
HPV infection is a risk factor in younger women

Symptoms

Vulvar cancer often presents with symptoms that may be mistaken for less serious conditions, leading to delays in diagnosis. It's crucial to be aware of the various signs and symptoms that may indicate vulvar cancer, particularly those that persist or worsen over time.

Primary Symptoms

Vulvar Itching

Persistent itching that doesn't respond to typical treatments and may be localized to one area of the vulva.

Pain or Tenderness

Discomfort in the vulvar area that may be constant or occur during activities like sitting or sexual intercourse.

Visible Changes

Lumps, ulcers, or growths on the vulva; changes in skin color or texture; thickened or raised patches.

Bleeding

Non-menstrual bleeding or discharge, bleeding after intercourse, or bleeding from vulvar lesions.

Associated Symptoms

Some patients may also experience:

Burning sensation in the vulvar area
Painful urination (dysuria)
Changes in the appearance of moles on the vulva
Swollen lymph nodes in the groin area
Fatigue or unexplained weight loss (in advanced cases)
Insomnia due to discomfort or anxiety
Emotional symptoms related to diagnosis
Back pain in advanced cases with metastasis

Important Warning Signs

Seek immediate medical attention if you notice any persistent vulvar changes, especially:

A lump or ulcer that doesn't heal within 2 weeks
Bleeding not related to menstruation
Severe pain or rapid changes in appearance
Any vulvar symptoms lasting more than 2 weeks

Causes

The exact cause of vulvar cancer is not fully understood, but researchers have identified several factors that contribute to the development of abnormal cells in the vulvar tissue. Like other cancers, vulvar cancer develops when normal cells undergo genetic mutations that cause them to grow and divide uncontrollably.

Primary Mechanisms

Vulvar cancer typically develops through one of two main pathways:

HPV-Related Pathway

This pathway is more common in younger women and involves:

Infection with high-risk human papillomavirus (HPV) types, particularly HPV 16 and 18
Development of vulvar intraepithelial neoplasia (VIN), a precancerous condition
Progression from VIN to invasive cancer over years or decades
Often associated with smoking, which impairs immune response to HPV

Non-HPV-Related Pathway

This pathway is more common in older women and involves:

Chronic inflammatory conditions of the vulva
Lichen sclerosus, a skin condition causing thinning and inflammation
Genetic mutations accumulating over time
Possible hormonal influences after menopause

Contributing Factors

Several factors may contribute to the development of vulvar cancer:

Chronic irritation: Long-term inflammation from various causes
Immunosuppression: Weakened immune system from HIV, medications, or other conditions
Previous cancer: History of cervical or vaginal cancer
Genetic factors: Family history of melanoma (for vulvar melanoma)
Environmental exposures: Though specific carcinogens are not well-established

Risk Factors

Understanding the risk factors for vulvar cancer can help identify women who may benefit from increased surveillance and preventive measures. While having risk factors doesn't mean cancer will develop, it's important to be aware of these associations.

Major Risk Factors

Age: Risk increases with age, with most cases occurring after age 65
HPV infection: Particularly high-risk types (16, 18, 31, 33)
Vulvar intraepithelial neoplasia (VIN): Precancerous changes in vulvar cells
Lichen sclerosus: A chronic skin condition affecting 4% of women
Smoking: Doubles the risk, especially in HPV-positive women

Additional Risk Factors

Immunosuppression: HIV infection, organ transplant recipients
Previous genital cancers: Cervical, vaginal, or anal cancer
Multiple sexual partners: Increases HPV exposure risk
Early age at first intercourse: Associated with HPV exposure
Chronic vulvar conditions: Including lichen planus
Northern European ancestry: Higher incidence rates observed

Protective Factors

Some factors may reduce the risk of vulvar cancer:

HPV vaccination before sexual activity
Regular gynecologic examinations
Not smoking or quitting smoking
Safe sexual practices
Treatment of precancerous conditions

Diagnosis

Early diagnosis of vulvar cancer significantly improves treatment outcomes. The diagnostic process typically involves multiple steps to confirm the presence of cancer, determine its type, and assess its extent.

Medical History and Physical Examination

The diagnostic process begins with:

Detailed medical history including symptoms, risk factors, and family history
Comprehensive pelvic examination
Visual inspection of the vulva under good lighting
Palpation to assess for lumps or abnormalities
Examination of groin lymph nodes

Diagnostic Procedures

Colposcopy

A specialized examination using a magnifying instrument to:

Examine vulvar tissue in detail
Identify abnormal areas for biopsy
Apply acetic acid or toluidine blue to highlight abnormal cells

Biopsy

The definitive diagnostic test involving:

Punch biopsy: Removal of small tissue samples
Excisional biopsy: Complete removal of small lesions
Incisional biopsy: Removal of part of larger lesions
Multiple biopsies may be needed for extensive disease

Staging Studies

Once cancer is confirmed, additional tests determine its extent:

Imaging studies: CT, MRI, or PET scans
Cystoscopy: Examination of bladder if involvement suspected
Proctoscopy: Examination of rectum if involvement suspected
Sentinel lymph node biopsy: To assess lymph node involvement
Chest X-ray: To check for lung metastases

Staging System

Vulvar cancer is staged using the FIGO (International Federation of Gynecology and Obstetrics) system:

Stage I: Cancer confined to vulva or perineum
Stage II: Cancer extends to lower urethra, vagina, or anus
Stage III: Cancer spread to regional lymph nodes
Stage IV: Advanced disease with distant spread

Treatment Options

Treatment for vulvar cancer depends on the cancer type, stage, location, and the patient's overall health. A multidisciplinary team approach involving gynecologic oncologists, radiation oncologists, and other specialists provides optimal care.

Surgical Treatment

Local Excision

For early-stage cancer:

Wide local excision with 1-2 cm margins
Preserves most vulvar tissue and function
May be combined with sentinel lymph node biopsy
Suitable for well-defined, small tumors

Radical Vulvectomy

For more extensive disease:

Partial: Removal of part of vulva
Complete: Removal of entire vulva
May include clitoris, labia, and surrounding tissue
Often combined with lymph node dissection
Reconstructive surgery may be offered

Lymph Node Management

Critical for staging and treatment:

Sentinel lymph node biopsy for early disease
Inguinofemoral lymphadenectomy for positive nodes
Bilateral dissection for midline tumors
May be done through separate incisions

Radiation Therapy

Primary Radiation

Used in specific situations:

Inoperable tumors
Patients unable to undergo surgery
Combined with chemotherapy (chemoradiation)
Typically involves external beam radiation

Adjuvant Radiation

After surgery to reduce recurrence risk:

Close or positive surgical margins
Multiple positive lymph nodes
Large primary tumors
May include vulva and/or groin areas

Systemic Therapy

Chemotherapy

Various roles in treatment:

Concurrent with radiation (radiosensitizer)
Neoadjuvant therapy to shrink tumors
Treatment of metastatic disease
Common drugs: cisplatin, 5-fluorouracil, mitomycin

Targeted Therapy and Immunotherapy

Emerging treatment options:

Bevacizumab for advanced disease
Pembrolizumab for PD-L1 positive tumors
Clinical trials investigating new agents
Personalized medicine approaches

Supportive Care

Comprehensive care includes:

Pain management
Wound care and lymphedema management
Sexual health counseling
Psychological support
Nutritional counseling
Physical therapy

Prevention

While not all cases of vulvar cancer can be prevented, several strategies can significantly reduce risk and enable early detection when treatment is most effective.

Primary Prevention

HPV Vaccination

Recommended for girls and boys ages 11-12
Can be given as early as age 9
Catch-up vaccination through age 26
May benefit some adults ages 27-45
Protects against cancer-causing HPV types

Lifestyle Modifications

Smoking cessation: Reduces risk by 50%
Safe sexual practices: Reduces HPV transmission
Limit sexual partners: Decreases HPV exposure
Maintain healthy immune system: Through diet and exercise

Secondary Prevention

Regular Screening

Annual gynecologic examinations
Self-examination monthly
Prompt evaluation of symptoms
Follow-up of abnormal findings

Management of Precancerous Conditions

Treatment of VIN (vulvar intraepithelial neoplasia)
Regular monitoring of lichen sclerosus
Appropriate therapy for chronic vulvar conditions
Biopsy of suspicious lesions

Self-Examination Guidelines

Monthly vulvar self-examination involves:

Using a hand mirror in good lighting
Examining all areas of the vulva
Looking for changes in color, texture, or appearance
Noting any new lumps, sores, or thickened areas
Reporting changes to healthcare provider promptly

When to See a Doctor

Early detection of vulvar cancer significantly improves treatment outcomes. Knowing when to seek medical attention is crucial for timely diagnosis and intervention.

Immediate Medical Attention

See a healthcare provider immediately if you experience:

A lump, ulcer, or sore on the vulva lasting more than 2 weeks
Bleeding from the vulva not related to menstruation
Severe vulvar pain or rapidly worsening symptoms
Changes in a mole on the vulva
Persistent itching not relieved by treatment

Red Flag Symptoms

These symptoms require urgent evaluation:

Any vulvar lesion in postmenopausal women
Non-healing vulvar wounds
Vulvar symptoms with enlarged groin lymph nodes
Persistent vulvar symptoms despite treatment

Routine Medical Care

Schedule regular appointments for:

Annual well-woman examinations
Follow-up of chronic vulvar conditions
Evaluation of new or changing symptoms
Screening if you have risk factors

What to Expect at Your Visit

Your healthcare provider will:

Take a detailed medical history
Perform a thorough pelvic examination
May use a colposcope for detailed viewing
Might recommend biopsy of suspicious areas
Order appropriate imaging if needed
Discuss findings and next steps

Related Conditions

Several conditions may be associated with, predispose to, or be mistaken for vulvar cancer. Understanding these relationships is important for proper diagnosis and management.

Precancerous Conditions

Vulvar Intraepithelial Neoplasia (VIN): Abnormal cell growth that may progress to cancer
Paget's Disease of the Vulva: Rare condition that can be associated with underlying cancer
Vulvar Melanoma in Situ: Early stage melanoma confined to skin surface

Chronic Vulvar Conditions

Lichen Sclerosus: Inflammatory condition causing white patches and thinning
Lichen Planus: Inflammatory condition causing erosions and scarring
Chronic Vulvitis: Long-term inflammation from various causes
Vulvar Psoriasis: Can cause red, scaly patches

Other Gynecologic Cancers

Cervical Cancer: Often shares HPV as a risk factor
Vaginal Cancer: May occur simultaneously or sequentially
Anal Cancer: Also associated with HPV infection
Ovarian Cancer: Different risk profile but may coexist

Benign Vulvar Lesions

Bartholin's Cysts: Fluid-filled cysts near vaginal opening
Vulvar Warts: Caused by low-risk HPV types
Sebaceous Cysts: Benign cysts from blocked glands
Vulvar Varicosities: Enlarged veins in vulvar area

Frequently Asked Questions

Is vulvar cancer hereditary?

Most vulvar cancers are not hereditary. However, there may be a slightly increased risk if you have a family history of melanoma (for vulvar melanoma) or certain genetic syndromes. The majority of cases are related to acquired risk factors like HPV infection, smoking, or chronic vulvar conditions rather than inherited genetic mutations.

Can vulvar cancer be cured?

Yes, vulvar cancer can often be cured, especially when detected early. The five-year survival rate for localized vulvar cancer (confined to the vulva) is over 85%. Even for regional disease (spread to nearby lymph nodes), the five-year survival rate is approximately 50-60%. Early detection and treatment are key to achieving the best outcomes.

Will treatment affect sexual function?

Treatment can impact sexual function, but the extent varies greatly depending on the type and extent of treatment. Many women can maintain satisfying sexual relationships after treatment. Options include: counseling, vaginal dilators, lubricants, reconstructive surgery, and working with a sexual health specialist. Open communication with your healthcare team is important.

How often does vulvar cancer recur?

Recurrence rates vary based on the initial stage and treatment. Local recurrence occurs in about 15-40% of cases, most commonly within the first two years after treatment. Regular follow-up examinations are crucial for early detection of recurrence. Risk factors for recurrence include positive margins, lymph node involvement, and advanced stage at diagnosis.

Can I prevent vulvar cancer if I have lichen sclerosus?

While lichen sclerosus increases vulvar cancer risk (about 4-5% lifetime risk), you can reduce this risk through: regular treatment with topical corticosteroids, routine monitoring by a healthcare provider, monthly self-examinations, avoiding irritants, maintaining good vulvar hygiene, and prompt evaluation of any changes. Most women with lichen sclerosus do not develop cancer.

What follow-up care is needed after treatment?

Follow-up care typically includes: physical examinations every 3-4 months for the first 2 years, then every 6 months for years 3-5, and annually thereafter. This may include pelvic exams, imaging studies as needed, and management of treatment side effects. The schedule may be adjusted based on individual risk factors and treatment response.

Vulvar Cancer