Carcinoma of the vulva

Introduction

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 to make the diagnosis.
  

General Considerations

The vast majority of cancers of the vulva are squamous lesions that classically have occurred in women over 50 years of age. Several subtypes (particularly 16, 18, and 31) of the human papillomavirus have been identified in some but not all vulvar cancers. As with squamous cell lesions of the cervix, a grading system of vulvar intraepithelial neoplasia (VIN) from mild dysplasia to carcinoma in situ has been established.

Differential Diagnosis

Biopsy is essential for the diagnosis of VIN and vulvar cancer 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.

Benign vulvar disorders that must be excluded in the diagnosis of carcinoma of the vulva include chronic granulomatous lesions (eg, lymphogranuloma venereum, syphilis), condylomas, hidradenoma, or neurofibroma. Lichen sclerosus and other associated leukoplakic changes in the skin should be biopsied. The likelihood that a superimposed vulvar cancer will develop in a woman with a nonneoplastic epithelial disorder (vulvar dystrophy) ranges from 1% to 5%.

Treatment

A. General Measures

Early diagnosis and treatment of irritative or other predisposing or contributing causes to carcinoma of the vulva should be pursued. 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, recommended treatment is 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.

B. Surgical Measures

High-grade VIN may be treated with a variety of approaches including topical chemotherapy, laser ablation, wide local excision, skinning vulvectomy, and simple vulvectomy. Small, invasive basal cell carcinoma of the vulva should be excised with a wide margin. If the VIN is extensive or multicentric, laser therapy or superficial surgical removal of vulvar skin may be required. In this way, the clitoris and uninvolved portions of the vulva may be spared.