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For further information, see CMDT Part 18-13: 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 vast majority of cancers of the vulva are squamous lesions; basal cell carcinomas are also found

  • Several subtypes (particularly 16, 18, and 31) of the human papillomavirus (HPV) have been identified in some but not all vulvar cancers

  • About 70–90% of vulvar intraepithelial neoplasia (VIN) and 40–60% of vulvar cancers are HPV associated

  • As with squamous cell lesions of the cervix, a grading system of VIN from mild dysplasia to carcinoma in situ is used

Demographics

  • Usually occurs in women over 50 years of age

  • The likelihood that a superimposed vulvar cancer will develop in a woman with a nonneoplastic epithelial disorder (vulvar dystrophy) is 1–5%

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, hidradenoma, 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

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