Vulvar cancers are uncommon and comprise approximately 5 percent of all gynecologic malignancies. Most vulvar cancers are diagnosed at an early stage (I and II). Advanced disease is found mainly in older women, perhaps due to clinical and behavioral barriers that lead to diagnostic delays. Thus, early detection of any abnormal vulvar lesion by biopsy is imperative to diagnosing this cancer in its early stages and improving subsequent morbidity and mortality rates.
Approximately 90 percent of vulvar tumors are squamous cell carcinomas (Fig. 31-1). Accordingly, virtually all knowledge regarding prognostic factors, spread patterns, and survival information is derived from women with this histologic type. Although rare, uncommon histologic subtypes such as melanomas, basal cell carcinomas, Bartholin gland adenocarcinomas, soft tissue sarcomas, and metastatic lesions may also be encountered (Table 31-1).
Vulvar squamous cell carcinoma. A. Low-power view. The surface epithelium shows high-grade squamous dysplasia. Nests of invasive squamous cell carcinoma (arrow) are present. A brisk chronic inflammatory infiltrate is present as is often the case with invasive squamous cell carcinoma. Portions of the surface epithelium extend deep and are cut tangentially (asterisks), giving the false impression of invasive tumor at these sites. B. Tumor shows classic diagnostic features of invasive squamous cell carcinoma that include a squamoid appearance, intercellular bridges, and brightly eosinophilic keratin pearls (arrows). Nests of invasive tumor are surrounded by chronic inflammation. (Photographs contributed by Dr. Kelley Carrick.)
Table 31-1. Histologic Subtypes of Vulvar Cancer |Favorite Table|Download (.pdf)
Table 31-1. Histologic Subtypes of Vulvar Cancer
Squamous cell carcinoma
Carcinoma of Bartholin gland
Transitional cell carcinoma
Vulvar Paget disease
Merkel cell tumors
Basal cell carcinoma
Vulvar malignant melanoma
Malignant fibrous histiocytoma
Malignant rhabdoid tumor
Metastatic cancers to vulva
Yolk sac tumors
In the United States, vulvar cancers carry a relatively good prognosis with a 5-year relative survival rate of 78 percent (Stroup, 2008). Traditional therapy includes radical excision of the vulva and inguinal lymphadenectomy. For advanced stages, adjuvant chemoradiation may be used preoperatively or postoperatively to aid tumor control.
Treatment of vulvar cancer frequently results in dramatic anatomic deformity that leads to significant negative effects on patient sexuality. However, during the past decade, management of vulvar cancer has trended toward more conservative surgery and improved psychosexual outcomes.
Vulvar cancer is primarily a disease of elderly women but has been observed in premenopausal women as well. In the United States, the age-adjusted incidence of invasive vulvar tumors has trended upward during the past three decades. This increase persists among all age groups and all geographic areas (Bodelon, 2009). Specifically, the age-adjusted incidence of vulvar carcinoma in situ ...