Very rare form of malignancy affecting the vulva

Aetiology

Risk factors

• Highest incidence in older women
• Vulval intraepithelial neoplasia (VIN) is considered a pre-malignant state
  • Young women - often multifocal, recurrent or persistent causing treatment problems
  • Older women - greater risk of progression to invasive squamous carcinoma
• Lichen sclerosus
• HPV infection
• Paget's disease of the vulva (adenocarcinoma in situ) and melanoma in situ are both pre-invasive conditions - both rare, but they have a significant risk of invasion

Pathophysiology

Histology of vulval carcinoma

• 85% of cancers of the vulva are squamous and the remaining are of various histological types, including melanomas

Vulval intraepithelial neoplasia (VIN)

• VIN of usual type is a precursor of HPV-driven SCC
• Differentiated VIN (dVIN) - precursor of HPV independent vulval SCC
  • Higher risk of malignancy then HPV-drive VIN
  • Often background of inflammatory dermatoses such as lichen sclerosus
• Often synchronous cervical and vaginal neoplasia
• Management: biopsy and resection

Clinical features

• Vulval cancer may present with a vulval lump, vulval bleeding due to ulceration, pruritus or pain
• 75% of all growths are primarily on the labia

Investigations