Vulval Carcinoma

Very rare form of malignancy affecing 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 condition - 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 presentation

  • 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

  • Examination and biopsy
  • Other investigations such as cystoscopy, proctoscopy, CXR and MRI scans are used for staging purposes

Management

  • Wide local excision of vulval lesion
    • If the depth of invasion is >1mm, the surgery includes inguinal lymphadenectomy (inguinal lymph node spread is an important prognostic factor)
  • Radiotherapy +/- chemotherapy is often used in the management of advanced vulval cancer