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.

Invasive carcinoma confined to the vulva without evidence of spread to adjacent organs or to the regional lymph nodes will necessitate radical vulvectomy and inguinal lymphadenectomy if the patient is able to withstand surgery. Debilitated patients may be candidates for palliative irradiation only.

Prognosis

Basal cell carcinomas very seldom metastasize, and carcinoma in situ by definition has not metastasized. With adequate excision, the prognosis for both lesions is excellent. Patients with invasive vulvar squamous cell carcinoma 3 cm in diameter or less without inguinal lymph node metastases who can sustain radical surgery have about a 90% chance of a 5-year survival. If the lesion is greater than 3 cm and has metastasized, the likelihood of 5-year survival is less than 25%.

Preferences:
Cardosi RJ et al: Diagnosis and management of vulvar and vaginal intraepithelial neoplasia. Obstet Gynecol Clin North Am 2001;28:685.

Hopkins MP et al: Carcinoma of the vulva. Obstet Gynecol Clin North Am 2001;28:791.