II. Epidemiology
- Most common over age 65 years
- Also affects <40 years (15% of cases)
- Associated with HPV Infection
III. Types
- Type I (Ages: 35 to 65 years)
- Poorly differentiated basaloid lesions
- Vulvar intraepithelial neoplasia (VIN)
- Pre-malignant lesion
- Associated with HPV infection (esp. 16, 18, 31)
- Tobacco Abuse is a predisposing factor
- Type II (Ages: 55 to 85 years)
- Well-differentiated squamous cell cancer
- Vulvar Non-neoplastic Epithelial Disorders (VNED)
- Vulvar inflammation
- Lichen Sclerosis
- Squamous cell hyperplasia
IV. Symptoms
- Vulvar Pruritus (most common)
- Vulvar bleeding or discharge
- Vulvar Pain
- Dysuria
V. Signs
- Raised exophytic vulvar lesion
- May be white or erythematous
- Most commonly affects labia majora
VI. Staging
- Stage 0 (Tis): Carcinoma in situ
- Stage I (T1 N0 M0): Confined to vulva/perineum (<2 cm)
- Stage II (T2 NO MO): Confined to vulva/perineum (>2 cm)
- Stage III (T1-3 N1 M0): Regional Lymph Node metastasis
- Stage IVA (T1-4 N2 M0): Pelvic metastasis
- Stage IVB (T1-4 N0-2 M1): Distant metastasis
VII. Differential Diagnosis: Other vulvar malignancies
- Paget Disease (<1% of vulvar malignancies)
- Melanoma (2% of vulvar malignancies)
- Bartholin's Gland Carcinoma (rare)
- Basal Cell Carcinoma of vulva (rare)
- Sarcoma of vulva (rare)
- Verrucous Carcinoma (rare)
VIII. Management
- Surgical excision
- Primary lesion removed with 1 cm margin
- Radical vulvectomy or
- Radical local excision
- Inguinal-femoral lymphadenectomy
- Indicated for >1 mm dermal invasion
- Primary lesion removed with 1 cm margin
- Postoperative groin and pelvic radiation
- Indicated for >2 nodes positive
IX. Prognosis: Five year survival
- Stage I: 98%
- Stage II: 85%
- Stage III: 74%
- Stage IV: 31%
- Positive pelvic nodes: 11%
X. References
- Muto in Ryan (1999) Kistner's Gynecology, p. 74-9
- Canavan (2002) Am Fam Physician 66(7):1269-76 [PubMed]
- Hopkins (2001) Obstet Gynecol Clin North Am 28(4):791 [PubMed]