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For further information, see CMDT Part 18-20: Carcinoma of the Vulva

Key Features

Essentials of Diagnosis

  • History of genital warts

  • History of prolonged vulvar irritation, with pruritus, local discomfort, or slight bloody discharge

  • Early lesions may suggest or include nonneoplastic epithelial disorders

  • Late lesions appear as a mass, an exophytic growth, or a firm, ulcerated area in the vulva

  • Biopsy is necessary for diagnosis

General Considerations

  • The majority of cancers of the vulva are squamous lesions that classically have occurred in women over 50 years of age

  • Vulvar low-grade squamous intraepithelial lesions (LSIL) are benign and do not require intervention

  • Vulvar high-grade squamous intraepithelial lesions (HSIL) and differentiated vulvar intraepithelial neoplasia (dVIN) are premalignant conditions

  • Vulvar HSIL (VIN usual type) is associated with HPV, while dVIN is associated with vulvar dermatoses, eg, lichens sclerosus

  • About 70–90% of premalignant lesions are vulvar HSIL, but HSIL is the precursor for only 20% of vulvar cancers, while dVIN is the precursor for approximately 80% of vulvar cancers

Clinical Findings

Symptoms and Signs

  • Early lesions may suggest or include nonneoplastic epithelial disorders

  • Late lesions appear as

    • A mass

    • An exophytic growth

    • A firm, ulcerated area in the vulva

Differential Diagnosis

  • Chronic granulomatous lesions (eg, lymphogranuloma venereum, syphilis), condylomas, or neurofibroma

  • Genital warts (condyloma acuminata)

  • Lichen sclerosus

  • Lichen planus

  • Ulcer: herpes simplex virus, chancroid, syphilis, granuloma inguinale, lymphogranuloma venereum, Behçet syndrome

  • Hidradenitis suppurativa

  • Vulvar intraepithelial neoplasia

  • Psoriasis

  • Paget disease

  • Papillary hidradenoma

  • Bartholin cyst or abscess

Diagnosis

Laboratory Tests

  • Pathologic examination of biopsies of lesion(s)

Imaging Studies

  • Preoperative colposcopy of vulva, vagina, and cervix

  • CT or MRI of the pelvis or abdomen is generally not required except in advanced cases for planning therapeutic options

Diagnostic Procedures

  • Biopsy is essential for the diagnosis and should be performed with any localized atypical vulvar lesion, including white patches

  • Multiple skin-punch specimens can be taken in the office under local anesthesia, with care to include tissue from the edges of each lesion sampled

  • Lichen sclerosus and other associated leukoplakic changes in the skin should be biopsied

Treatment

Medications

  • A 7:3 combination of betamethasone and crotamiton is particularly effective for itching

  • After an initial response, fluorinated steroids should be replaced with hydrocortisone because of their skin atrophying effect

  • For lichen sclerosus

    • Apply clobetasol propionate cream 0.05% twice daily for 2–3 weeks, then once daily until symptoms resolve

    • Application one to three times a week can be used for long-term maintenance therapy

Surgery

  • In situ carcinoma

    • In ...

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