Vulval Cancer – Comprehensive Medical Guide

Overview

Vulval cancer is a malignant growth that originates in the external female genitalia, known as the vulva. The vulva includes the labia majora, labia minora, clitoral hood, clitoris, urethral opening, and the opening of the vagina. Most vulval cancers are squamous cell carcinomas, arising from the flat, scale‑like cells that line the surface of the vulva, but other histologic types (melanoma, adenocarcinoma, basal cell carcinoma) can occur.

• Who it affects: Primarily women over age 60, but it can occur at any age, including in adolescents (especially the rarer melanoma type).
• Prevalence: In the United States, vulval cancer accounts for about 0.5% of all cancers diagnosed in women—approximately 6,130 new cases and 1,160 deaths per year (American Cancer Society, 2024). Worldwide incidence varies, ranging from 1–2 cases per 100,000 women in high‑income countries to slightly higher rates in low‑resource settings where HPV infection is common.

Because vulval cancer is relatively rare, many women and even some health‑care providers may not recognize early signs. Early detection dramatically improves outcomes, with 5‑year survival exceeding 80% for localized disease but falling below 40% for advanced or metastatic disease ^{[CDC 2023]}.

Symptoms

Symptoms often develop slowly and may be mistaken for benign conditions such as yeast infections or dermatitis. Any persistent change deserves evaluation.

• Visible lesion or lump: A raised, thickened, or ulcerated area on the vulva that does not heal within 2–3 weeks.
• Itching (pruritus): Persistent or worsening itch that may be accompanied by burning.
• Pain: Discomfort or sharp pain in the vulvar region, especially during intercourse (dyspareunia) or sitting.
• Bleeding or discharge: Unexplained spotting, bleeding after intercourse, or a watery/serous discharge.
• Changes in skin color or texture: Redness, white patches (lichenoid changes), or areas that look “velvety.”
• Swelling or lumps in the groin: Enlargement of the inguinal lymph nodes may indicate spread.
• Odor: A foul smell coming from the vulva or vaginal area.
• Difficulty urinating: If a tumor blocks the urethral opening.

Symptoms are usually unilateral (affect one side) but can involve both sides. If any of these signs persist for more than three weeks, schedule an appointment with a health‑care professional.

Causes and Risk Factors

Most vulval cancers are linked to DNA damage caused by infections, chronic inflammation, or smoking. Below is a summary of known contributors.

Human Papillomavirus (HPV)

High‑risk HPV types 16 and 18 are found in 40–50% of vulvar squamous cell carcinomas, especially in younger women (<50 y). The virus integrates into cellular DNA, prompting malignant transformation ^{[NIH 2020]}.

Precancerous lesions

• Vulvar intraepithelial neoplasia (VIN): Particularly the usual type (uVIN) linked to HPV.
• Lichen sclerosus: A chronic inflammatory condition that can lead to differentiated VIN and subsequent cancer, especially in older women.

Other risk factors

• Age > 60 years.
• Smoking – contains carcinogens that affect vulvar epithelium.
• Immunosuppression – organ transplant recipients, HIV infection.
• History of cervical, anal, or vaginal cancer.
• Chronic vulvar irritation (e.g., poor hygiene, chronic dermatitis).
• Family history of gynecologic cancers (BRCA1/2 mutations may increase risk, though evidence is limited).

Diagnosis

Diagnosing vulval cancer requires a combination of visual examination, tissue sampling, and imaging to assess spread.

Clinical Examination

• Full gynecologic inspection with a speculum and gentle palpation of the vulva and inguinal lymph nodes.
• Documentation of lesion size, location, and appearance (photography may be used for monitoring).

Biopsy

The definitive test. Options include:

• Punch or shave biopsy: Most common for small lesions.
• Incisional biopsy: Larger tissue sample for extensive lesions.
• Excisional biopsy: Removal of the entire lesion when feasible.

Pathology will identify the cancer type, grade, depth of invasion, and presence of HPV DNA.

Imaging Studies

• Pelvic MRI: Best for local staging, evaluating depth of invasion, and relation to adjacent structures.
• CT scan of the abdomen/pelvis: Detects distant spread (lung, liver).
• PET‑CT: Sensitive for lymph node involvement and metastases.
• Ultrasound of inguinal nodes: Simple bedside tool for evaluating palpable groin nodes.

Staging

Staging follows the FIGO (International Federation of Gynecology and Obstetrics) system, ranging from Stage 0 (in‑situ) to Stage IV (metastatic). Accurate staging guides treatment choice.

Treatment Options

Treatment is individualized based on stage, tumor size, location, patient health, and personal preferences. Multidisciplinary care (gynecologic oncologist, radiation oncologist, medical oncologist, urogynecologist, psycho‑social support) yields the best outcomes.

Surgery

• Wide local excision (WLE): Removal of the tumor with a 1–2 cm margin of healthy tissue; preferred for early-stage disease.
• Partial or total vulvectomy: Reserved for larger or deeply invasive tumors.
• Inguinal lymphadenectomy: Removal of groin lymph nodes; can be done via an open approach or sentinel‑node biopsy (less morbidity).
• Reconstructive procedures: Skin grafts, flaps, or laser therapy to restore function and appearance.

Potential surgical complications include wound infection, lymphedema, and sexual dysfunction.

Radiation Therapy

• External beam radiation (EBRT) to the vulva and pelvic nodes.
• Often combined with chemotherapy (chemoradiation) for locally advanced disease (Stage III‑IV).
• Can be used as adjuvant treatment after surgery when margins are close or nodes are positive.

Chemotherapy

Primarily used in conjunction with radiation (cisplatin‑based regimens) for advanced disease. For metastatic or recurrent cancer, systemic therapy may include:

• Cisplatin + 5‑Fluorouracil (5‑FU).
• Taxanes (paclitaxel or docetaxel).
• Targeted agents (e.g., pembrolizumab for PD‑L1‑positive tumors) – emerging evidence from clinical trials.

Immunotherapy

Checkpoint inhibitors have shown activity in vulvar melanoma and HPV‑related cancers. FDA approved pembrolizumab for PD‑L1‑positive advanced vulvar cancers (2022) ^{[FDA 2022]}.

Lifestyle & Supportive Care

• Smoking cessation to improve healing and reduce recurrence risk.
• Pain management (NSAIDs, neuropathic agents, pelvic floor physical therapy).
• Psychosocial counseling—address body image, sexual health, and anxiety.
• Nutrition: high‑protein diet to support wound healing.

Living with Vulval Cancer

Beyond active treatment, day‑to‑day management focuses on comfort, emotional well‑being, and prevention of complications.

Physical Care

• Hygiene: Use mild, fragrance‑free cleansers; pat dry rather than rub.
• Clothing: Loose, breathable cotton underwear to reduce moisture and irritation.
• Pelvic floor therapy: Helps with pain, urinary symptoms, and sexual function.
• Lymphedema monitoring: Gentle manual lymph drainage if nodes were removed.

Emotional & Social Support

• Join support groups (e.g., Gilda's Club, Cancer Support Community).
• Consider counseling for depression or anxiety, which affect up to 30% of cancer patients ^{[Cleveland Clinic 2023]}.
• Open communication with partner about intimacy; use lubricants and explore non‑penetrative activities if needed.

Follow‑Up Schedule

Typical follow‑up after definitive treatment:

• Every 3–4 months for the first 2 years (exam, symptom review, imaging as indicated).
• Every 6 months for years 3‑5.
• Annual visits thereafter.

Promptly report new symptoms such as lump, ulceration, or unexplained pain.

Prevention

Because many risk factors are modifiable, prevention strategies can lower incidence.

• HPV vaccination: Gardasil 9 protects against HPV 16/18 and other high‑risk types; CDC recommends routine vaccination for girls and boys at ages 11–12, and catch‑up through age 26 (and shared‑decision up to age 45). Studies show a >70% reduction in vulvar VIN after vaccination ^{[Mayo Clinic 2019]}.
• Practice safe sex (condom use reduces HPV transmission).
• Quit smoking – each pack‑year adds roughly a 1.5‑fold increase in vulvar cancer risk.
• Manage chronic vulvar conditions (e.g., treat lichen sclerosus with high‑potency topical steroids).
• Routine gynecologic exams: early detection of precancerous changes.

Complications

If left untreated or if treatment is delayed, vulval cancer can lead to serious health problems.

• Local invasion: Into the urethra, vagina, or rectum causing urinary or bowel obstruction.
• Metastasis: Spread to inguinal nodes, pelvis, lungs, liver, or bone.
• Chronic pain: Neuropathic pain affecting quality of life.
• Lymphedema: Swelling of the thigh/groin after lymph node removal.
• Sexual dysfunction: Pain, loss of sensation, or psychological distress.
• Psychological impact: Depression, anxiety, and body‑image issues.

When to Seek Emergency Care

References

1. American Cancer Society. Cancer Facts & Figures 2024. https://www.cancer.org/research/cancer-facts-statistics.html
2. Centers for Disease Control and Prevention. Vulvar Cancer. 2023. https://www.cdc.gov/cancer/vulvar/
3. World Health Organization. Human papillomavirus (HPV) and cancer. 2022.
4. Mayo Clinic. HPV vaccine: Facts and Side Effects. 2019. https://www.mayoclinic.org/healthy-lifestyle/young-adult/in-depth/hpv-vaccine/art-20456649
5. National Institutes of Health. Vulvar intraepithelial neoplasia and cancer. 2020. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6905476/
6. Cleveland Clinic. Psychological Effects of Cancer. 2023. https://my.clevelandclinic.org/health/diseases/20068-psychological-effects-of-cancer
7. U.S. Food and Drug Administration. Pembrolizumab (Keytruda) FDA Approval. 2022.