Vulvar carcinoma should be suspected in a woman with complaints of long-standing duration or those that do not resolve with standard treatment. Although mostly a disease of postmenopausal women, there has been a trend toward younger age at diagnosis. Many patients will attempt to ignore symptoms or to engage in a variety of self-treatments prior to presentation. Many physicians will treat lesions with topical steroids or estrogens prior to consideration of biopsy. The old adage, ''if cancer is the question, tissue is the issue,'' holds most true for the vulva and early biopsy of suspicious lesions is advised. Lesions can be ulcerated, nodular, or superficially spreading. A finding of microangiogenesis with mosaicism or any other abnormal vascular pattern is particularly suspicious.
In a review of the clinical events preceding a diagnosis of squamous cell carcinoma of the vulva, Jones and Joura noted that 88% experienced symptoms for more than six months, 31% had three or more medical consultations, and 27% had applied topical estrogens or topical steroids (9). Luckily, these cancers are relatively rare, comprising less than 5% of all gynecologic cancers. As expected, the majority of vulvar cancers are squamous in histology. However, malignant melanoma is the second most common cancer type. Other histologic subtypes are basal cell, adenocar-cinomas, and a variety of soft-tissue sarcomas—leiomyosarcomas, angiosarcomas, rhabdomyosarcomas, epithelioid carcinomas, and Kaposi's sarcomas. A number of pelvic and genital tumors such as cancers of the uterus (urethral metastases), bladder, vagina, cervix and anorectum, and others can metastasize to the vulva. For this reason, women with pelvic and lower genital tract cancers need to be monitored for coexistent lesions in the vulva and vice versa.
Interestingly, HPV is seen in only 10% to 50% of invasive lesions as compared to 90% of invasive cervical cancers. There seem to be two distinct histologic subtypes. One is associated with HPV and has warty or basaloid features, and the other, keratinizing squamous carcinomas, is not HPV related. Both vulvar intraepithelial neoplasia and chronic vulvar inflammatory lesions are associated with higher rates of progression to invasive disease.
Treatment is individualized and can involve a combination of surgery, chemora-diation, and reconstructive techniques. There is a trend to treat early lesions with local deep resection with unilateral inguinal node dissection. Advanced lesions confined locally can be managed by neoadjuvant chemoradiation with surgical excision and reconstructive flap placement. Treatment of advanced metastatic disease is palliative.
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