II. Epidemiology

  1. Most common over age 65 years
  2. Also affects <40 years (15% of cases)
    1. Associated with HPV Infection

III. 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

IV. Symptoms

  1. Vulvar Pruritus (most common)
  2. Vulvar bleeding or discharge
  3. Vulvar Pain
  4. Dysuria

V. Signs

  1. Raised exophytic vulvar lesion
    1. May be white or erythematous
  2. Most commonly affects labia majora

VI. 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

VII. 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)

VIII. 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

IX. 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%

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