Vulvar Carcinoma

Vulvar Carcinoma
Photo by National Cancer Institute / Unsplash
  • Rare gynecologic cancer (<5% of GYN malignancies)
  • Most common type: Squamous cell carcinoma (~90%)
  • Two main pathways:
  • HPV-associated (younger women)
    • Types 16, 18
    • Risk factors: multiple sexual partners, early sexual activity, smoking, immunosuppression
    • Often associated with vulvar intraepithelial neoplasia (VIN)
  • Non-HPV-associated (older women)
    • Usually postmenopausal
    • Associated with chronic inflammatory conditions:
      • Lichen sclerosus
        • Most common in postmenopausal women. Thought to be autoimmune
        • Can affect vulva, perianal area, or other skin
        • Porcelain-white plaques with atrophy ("cigarette paper" texture)
        • Figure-8 or keyhole distribution (vulvar + perianal)
        • Intense pruritus, burning, dyspareunia
        • Scarring, loss of labia minora, narrowed introitus
      • Lichen planus
        • Inflammatory disorder involving vaginal mucosa, oral mucosa, and vulva
        • Purple, polygonal, pruritic papules (on skin)
        • Erosive type most common on vulva/vagina:Glazed, bright red erosions with white lacy borders (Wickham striae)Vaginal discharge, vaginal adhesions (synechiae)Pain, burning, dyspareunia
        • Oral mucosa: white reticulated plaques (also Wickham striae)

Risk Factors

  • HPV infection (types 16, 18)
  • Smoking
  • Immunosuppression (HIV, transplant)
  • Chronic vulvar irritation or dermatoses
  • History of cervical or vaginal neoplasia

Clinical Features

  • Chronic vulvar itching (most common symptom)
  • Vulvar pain, burning, or bleeding
  • Visible lesion:
    • White, red, or pigmented plaque
    • Ulcerative or exophytic mass (especially on labia majora)
  • Inguinal lymphadenopathy (advanced disease)

Diagnosis

  • Vulvar biopsy
    • Don’t delay-treat any chronic lesion or change suspiciously
  • Colposcopic evaluation of vulva may assist in margin assessment
  • Workup includes:
    • Pap smear
    • Colposcopy
    • HIV testing (if < 40 or immunocompromised)

Histologic Types

  • Squamous cell carcinoma (most common)
  • Melanoma (2nd most common)
  • Others: Adenocarcinoma, Basal cell carcinoma, Sarcoma

Staging (FIGO)

  • Stage I: Confined to vulva
  • Stage II: Extension to adjacent structures (e.g., lower urethra, vagina, anus)
  • Stage III: Involvement of inguinofemoral lymph nodes
  • Stage IV: Distant structures (upper vagina, urethra, rectum)

Management

  • Depends on stage and extent
  • Early-stage (localized disease):
    • Wide local excision or radical vulvectomy
    • Sentinel lymph node biopsy or inguinofemoral lymphadenectomy
  • Advanced-stage:
    • Radical surgery + radiation therapy
    • Chemoradiation may be used in inoperable cases or for nodal disease