Vulval cancer

Definition
Vulval cancer is a malignant tumour arising from the skin or mucosa of the vulva. It is a relatively uncommon gynaecological cancer, most often affecting the labia majora and labia minora, and occurs mainly in older women, though HPV-associated cases are increasingly seen in younger patients.

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Types

• Squamous cell carcinoma (SCC) – ~90% of cases

  • Keratinising SCC – usually in older women, often associated with lichen sclerosus or chronic inflammatory dermatoses

  • Basaloid or warty SCC – more common in younger women, associated with high-risk HPV (types 16, 18, 33)

• Others (rare)

  • Melanoma

  • Basal cell carcinoma

  • Bartholin gland adenocarcinoma

  • Paget’s disease of the vulva

  • Sarcomas

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Risk Factors

• Persistent infection with high-risk HPV (especially 16, 18, 33)

• Vulval intraepithelial neoplasia (VIN)

• Chronic inflammatory vulval skin disorders (lichen sclerosus, lichen planus)

• Increasing age (peak incidence >70 years)

• Immunosuppression (HIV, post-transplant)

• Smoking

• Previous cervical or vaginal cancer or high-grade intraepithelial neoplasia

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Symptoms

• Persistent vulval itching (most common early symptom)

• Vulval lump, ulcer, or thickened area

• Pain, burning, or tenderness

• Non-healing sore or bleeding lesion

• In advanced disease: inguinal lymphadenopathy, urinary or bowel symptoms

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Diagnosis

• Clinical examination: inspection and palpation of vulva, inguinal lymph nodes, and pelvic exam

• Biopsy: essential for histological diagnosis (incisional or punch biopsy from lesion edge)

• Imaging: MRI pelvis, CT chest/abdomen/pelvis, PET-CT for staging

• Staging: FIGO system (I–IV) based on tumour size, depth of invasion, and spread to lymph nodes or distant organs

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Treatment
Management depends on stage, histology, and patient factors, and is best handled in a multidisciplinary cancer team.

1. Early-stage disease

   • Wide local excision or radical local excision with adequate margins

   • Sentinel lymph node biopsy or inguinal lymphadenectomy if indicated

2. Locally advanced disease

   • Radical vulvectomy with bilateral inguinal–femoral lymphadenectomy

   • Adjuvant radiotherapy (± chemotherapy) for node-positive or margin-positive disease

3. Unresectable or advanced disease

   • Chemoradiation as primary treatment

   • Palliative radiotherapy or systemic therapy for symptom control

4. Special histologies

   • Vulval melanoma: wide local excision, sentinel lymph node biopsy, possible immunotherapy

   • Paget’s disease: wide excision, monitor for underlying adenocarcinoma

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Follow-up

• Regular clinical examination (every 3–6 months for first 2 years, then every 6–12 months up to 5 years)

• Surveillance for recurrence, management of treatment side effects, psychological and sexual support

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Prognosis

• Early-stage disease: 5-year survival ~80–90%

• Advanced-stage disease with nodal involvement: survival falls to ~40–50%

• Prognosis depends on tumour stage, lymph node status, and margin status

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Prevention and Risk Reduction

• HPV vaccination to reduce HPV-associated vulval cancers

• Smoking cessation

• Regular follow-up for women with VIN or lichen sclerosus

• Early investigation of persistent vulval symptoms