HemeOnc

Vulvar Cancer

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Vulvar Cancer, Carcinoma of the vulva

  • Epidemiology
  1. Most common over age 65 years
  2. Also affects <40 years (15% of cases)
    1. Associated with HPV Infection
  • Types
  1. Type I (Ages: 35 to 65 years)
    1. Poorly differentiated basaloid lesions
    2. Vulvar intraepithelial neoplasia (VIN)
      1. Pre-malignant lesion
      2. Associated with HPV infection (esp. 16, 18, 31)
      3. Tobacco abuse is a predisposing factor
  2. Type II (Ages: 55 to 85 years)
    1. Well-differentiated squamous cell cancer
    2. Vulvar Non-neoplastic Epithelial Disorders (VNED)
      1. Vulvar inflammation
      2. Lichen Sclerosis
      3. Squamous cell hyperplasia
  • Symptoms
  1. Vulvar Pruritus (most common)
  2. Vulvar bleeding or discharge
  3. Vulvar Pain
  4. Dysuria
  • Signs
  1. Raised exophytic vulvar lesion
    1. May be white or erythematous
  2. Most commonly affects labia majora
  • Staging
  1. Stage 0 (Tis): Carcinoma in situ
  2. Stage I (T1 N0 M0): Confined to vulva/perineum (<2 cm)
  3. Stage II (T2 NO MO): Confined to vulva/perineum (>2 cm)
  4. Stage III (T1-3 N1 M0): Regional lymph node metastasis
  5. Stage IVA (T1-4 N2 M0): Pelvic metastasis
  6. Stage IVB (T1-4 N0-2 M1): Distant metastasis
  • Differential Diagnosis
  • Other vulvar malignancies
  1. Paget Disease (<1% of vulvar malignancies)
  2. Melanoma (2% of vulvar malignancies)
  3. Bartholin's Gland Carcinoma (rare)
  4. Basal Cell Carcinoma of vulva (rare)
  5. Sarcoma of vulva (rare)
  6. Verrucous Carcinoma (rare)
  • Management
  1. Surgical excision
    1. Primary lesion removed with 1 cm margin
      1. Radical vulvectomy or
      2. Radical local excision
    2. Inguinal-femoral lymphadenectomy
      1. Indicated for >1 mm dermal invasion
  2. Postoperative groin and pelvic radiation
    1. Indicated for >2 nodes positive
  • Prognosis
  • Five year survival
  1. Stage I: 98%
  2. Stage II: 85%
  3. Stage III: 74%
  4. Stage IV: 31%
  5. Positive pelvic nodes: 11%