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BASICS

  • VIN = premalignant vulvar lesions; >70% linked to HPV.
  • Squamous cell carcinoma = most common invasive vulvar malignancy (90%).
  • Melanoma second most common (8%), sarcoma third.
  • Other rare malignancies: basal cell carcinoma, Paget disease, Bartholin gland adenocarcinoma, small cell carcinoma, verrucous carcinoma.
  • Sarcomas usually leiomyosarcomas, arise at round ligament insertion or other vulvar structures.
  • Rarely ectopic breast carcinoma occurs in vulva.

EPIDEMIOLOGY

  • Estimated 6,020 new cases and 1,150 deaths (2017 data).
  • Mean age at diagnosis: 65 years overall.
  • Mean age: 40 years for in situ disease; 60 years for invasive malignancy.

ETIOLOGY AND PATHOPHYSIOLOGY

  • HPV associated with ~55% of vulvar cancers, predominantly HPV types 16 and 33.
  • Two pathways for squamous cell carcinoma:
  • HPV-related (warty/basaloid type, younger women)
  • Non-HPV-related (lichen sclerosus pathway, older women)
  • Smoking contributes by direct vulvar exposure or systemic carcinogen absorption.
  • Patients with cervical cancer have increased risk for vulvar cancer.

RISK FACTORS

  • History of VIN or cervical intraepithelial neoplasia (CIN).
  • Smoking.
  • Lichen sclerosus (vulvar dystrophy).
  • HPV infection, condylomata, other STDs.
  • Low socioeconomic status.
  • Autoimmune diseases.
  • Immunodeficiency.
  • Northern European ancestry.
  • Risk factors for recurrence: age >50, positive excision margins, concurrent vaginal intraepithelial neoplasia (VAIN).

GENERAL PREVENTION

  • HPV vaccination can reduce vulvar cancer by up to 60%.
  • Smoking cessation counseling.

COMMONLY ASSOCIATED CONDITIONS

  • Elderly patients often have comorbid medical conditions.
  • High rate of other gynecologic malignancies.

DIAGNOSIS

History

  • Pruritus or raised lesion in vulvar area.
  • Vaginal bleeding or discharge.

Physical Exam

  • In situ disease: small raised lesion, plaque, ulcer or mass commonly on labia majora.
  • Invasive disease: ulcerated, nonhealing lesion with pain, bleeding, foul discharge.
  • Enlarged inguinal lymph nodes in advanced disease.

Differential Diagnosis

  • Infectious ulcers (syphilis, lymphogranuloma venereum, granuloma inguinale).
  • Bartholin gland disorders, seborrheic keratosis, hidradenomas.
  • Lichen sclerosus, epidermal inclusion cysts.
  • Crohn disease vulvar involvement.
  • Metastases to vulva.

Diagnostic Tests

  • Biopsy mandatory for any suspicious lesion.
  • Acetic acid wash with colposcopy for acetowhite/vascular lesions.
  • Pap smear and colposcopy of cervix, vagina, and vulva.
  • CT for lymph node and metastasis evaluation if tumor >2 cm or suspected metastasis.
  • Squamous cell antigen may be elevated in invasive disease.
  • Hypercalcemia may indicate metastasis.

STAGING (FIGO)

  • Stage I: confined to vulva
  • IA: ≤2 cm, ≤1 mm stromal invasion, no nodes
  • IB: >2 cm or >1 mm invasion, no nodes
  • Stage II: extends to adjacent perineal structures (lower urethra, vagina, anus), no nodes
  • Stage III: any size tumor with positive inguinofemoral lymph nodes
  • IIIA, IIIB, IIIC based on number and size of nodes and extracapsular spread
  • Stage IV: invades upper urethra/vagina, bladder, rectum, or distant metastases

TREATMENT

General Measures

  • Wide excision for carcinoma in situ.
  • Cystoscopy/sigmoidoscopy if invasion suspected.
  • Neoadjuvant chemotherapy under investigation.
  • Chemoradiotherapy for advanced disease.
  • Radiation contraindicated for verrucous carcinoma.

Surgery

  • Wide excision or laser vaporization for in situ.
  • Radical local excision with lymphadenectomy based on stage.
  • Sentinel lymph node biopsy for early invasive lesions.
  • Pelvic exenteration for advanced cases invading bladder/rectum.
  • Radical vulvectomy with separate incisions preferred for cosmesis.

Radiation and Chemotherapy

  • Preoperative radiation may reduce surgery extent.
  • Adjuvant radiation for large tumors, positive margins, lymphovascular invasion, or node involvement.
  • Concurrent cisplatin-based chemoradiation is standard for advanced disease.

ADMISSION AND NURSING

  • Monitor for wound healing; ~50% experience wound breakdown.
  • Lymphedema in 15-20%; support hose and leg elevation advised.
  • Monitor for urinary incontinence and psychosexual effects.

ONGOING CARE

  • Follow-up exams every 6 months (early stage) or every 3 months (advanced) for first 2 years.
  • Annual cervical/vaginal cytology.
  • Majority relapses occur within first year.

PATIENT EDUCATION

  • Educate about HPV vaccination.
  • Emphasize importance of biopsy for suspicious lesions.
  • Discuss prognosis based on stage and lymph node involvement.

PROGNOSIS

  • 5-year survival:
  • Stage I: 78.5%
  • Stage II: 58.8%
  • Stage III: 43.2%
  • Stage IV: 13.0%
  • Lymph node involvement strongest survival predictor.

COMPLICATIONS

  • Wound breakdown and infection.
  • Lymphedema.
  • Urinary stress incontinence.
  • Psychosexual dysfunction.

REFERENCES

  1. Hinten F, Molijn A, Eckhardt L, et al. Vulvar cancer: two pathways with different precursors and genetic make-up. Gynecol Oncol. 2018;149(2):310-317.

ICD10

  • C51.9 Malignant neoplasm of vulva, unspecified
  • D07.1 Carcinoma in situ of vulva
  • C51.0 Malignant neoplasm of labium majus

Clinical Pearls

  • 55% of vulvar cancers are HPV-related; VAIN and AIN are also attributable to HPV.

  • HPV vaccination can reduce vulvar cancer by one-third.
  • Always biopsy suspicious or nonhealing vulvar lesions.