Vulvar Cancer

Vulvar cancer is a malignancy of the vulva — the external female genitalia including the labia, clitoris, and perineum. Most vulvar cancers are squamous cell carcinomas arising from the epithelial cells of the skin. Vulvar cancer is unusual in that it actually represents two different disease pathways with different causes, different age groups affected, and potentially different treatment approaches. [ACS, 2024]

The two main pathways are:

• HPV-related vulvar cancer — associated with HPV infection, typically occurs in younger women (35-55 years), often presents as vulvar intraepithelial neoplasia (VIN) that may progress to invasive cancer
• Non-HPV-related vulvar cancer — associated with chronic skin conditions like lichen sclerosus and lichen planus, typically occurs in older women (70+ years), often presents de novo without a precursor lesion

Understanding which pathway applies to your cancer helps your care team optimize treatment and counseling. Early recognition of symptoms and lesions can lead to earlier, more conservative treatment and better outcomes.

HPV Infection

Persistent infection with high-risk HPV types (especially HPV-16) significantly increases vulvar cancer risk, particularly in younger women. The same HPV types that cause cervical cancer can cause vulvar and anal cancers. [ACS, 2024]

Lichen Sclerosus

A chronic inflammatory skin condition causing itching and skin changes. Associated with HPV-negative vulvar cancer, typically in older women. About 4-5% of women with lichen sclerosus develop vulvar cancer over their lifetime. [NCI, 2024]

Lichen Planus

Another chronic inflammatory dermatologic condition that increases vulvar cancer risk. Erosive lichen planus particularly increases risk. [ACS, 2024]

Smoking

Smoking increases vulvar cancer risk, particularly HPV-related vulvar cancer. This may be related to reduced immune function and persistent HPV infection. [NCI, 2024]

Immunosuppression

HIV-positive women, organ transplant recipients, and others with weakened immune systems have increased risk of HPV-related vulvar cancer and precancerous lesions. [ACS, 2024]

Age

HPV-related vulvar cancer peaks in women aged 35-55. Non-HPV vulvar cancer associated with chronic skin disease peaks in women over 70. Median age at diagnosis is 65-70 years. [ACS, 2024]

Pelvic Exam & Visual Inspection

Your gynecologist will visually examine the vulva and feel for any masses or lymph node enlargement. If a lesion is visible, biopsy is the next step. [ACS, 2024]

Biopsy & Pathology

A small tissue sample is taken from the suspicious area under local anesthesia and examined under a microscope. This confirms the diagnosis, determines histology (squamous cell carcinoma, melanoma, etc.), and identifies HPV status. Biopsy is the gold standard for diagnosis. [NCI, 2024]

Colposcopy

Magnified visualization of the vulva to identify exact location of lesions and guide biopsy sampling, particularly useful if multiple lesions are present. [ACS, 2024]

Imaging: CT or MRI

Once invasive cancer is diagnosed, CT or MRI imaging helps determine if cancer has spread to lymph nodes or distant sites. Pelvic/inguinal lymph node assessment is critical for staging. [NCI, 2024]

HPV & Molecular Testing

HPV testing on the biopsy specimen helps classify the cancer as HPV-positive or HPV-negative, which guides treatment decisions and prognosis counseling. [ACS, 2024]

Laser Therapy & Topical Treatment

For early VIN or small lesions, laser ablation (usually CO2 laser) can destroy precancerous tissue with minimal scarring. Topical agents like imiquimod (immune stimulator) or 5-FU are increasingly used for VIN management, avoiding surgery. [NCCN, 2024]

Wide Local Excision (WLE)

Surgical removal of the tumor with a margin of normal tissue (typically 5-10 mm) while preserving as much vulvar tissue as possible. For early-stage invasive cancers (Stage IA), WLE alone is often adequate. Closure is typically primary (stitched immediately), avoiding need for skin grafts. [ACS, 2024]

Mohs Micrographic Surgery

Specialized surgical technique allowing margin assessment during surgery. Removes tumor with real-time histologic guidance, allowing for tissue-sparing treatment. Increasingly used in vulvar cancer for margin-controlled excision. [NCI, 2024]

Inguinofemoral Lymphadenectomy

Removal of inguinal and femoral lymph nodes on one or both sides. Essential for staging intermediate and advanced vulvar cancer. Sentinel lymph node biopsy (removing only the first lymph nodes cancer reaches) is increasingly used in early-stage disease to minimize morbidity. [NCCN, 2024]

Radical Vulvectomy

Removal of the entire vulva (labia majora, labia minora, clitoris, and surrounding tissue). Historically standard for invasive vulvar cancer. Now reserved for extensive or multifocal disease, as most women can be treated with WLE. [ACS, 2024]

Chemotherapy & Radiation

Neoadjuvant chemotherapy (chemoradiation with cisplatin and 5-FU) followed by surgery can be used for advanced disease, reducing tumor burden before surgery. Adjuvant radiation after surgery is used for high-risk features or inadequate surgical margins. [NCCN, 2024]

Follow-Up Surveillance

Regular gynecologic exams every 3 months for the first 2 years, then less frequently. Watch for recurrent lesions or lymphedema. Imaging is used as clinically indicated. [NCCN, 2024]

Sexual & Pelvic Health

Discuss sexual function concerns with your gynecologist. Vaginal dilators, lubricants, and pelvic floor physical therapy help address functional changes. Many women benefit from counseling about body image and sexuality after treatment. [ACS, 2024]

Lymphedema Management

If you had lymphadenectomy, work with a lymphedema specialist. Compression garments, massage therapy, and elevation help prevent and manage swelling. Early intervention is key. [NCI, 2024]

Emotional Support

Diagnosis and treatment of vulvar cancer can affect self-image, sexuality, and emotional wellbeing. Counseling, support groups, and psychiatric care when needed are integral to care. Many patients report anxiety about recurrence. [ACS, 2024]