Abstract: |
Vulvar cancer is the rarest gynecologic malignancy, accounting for approximately 6000 new cases and 1350 deaths annually. This represents approximately 0.3% lifetime risk of vulvar cancer among American women. The rate of vulvar cancer diagnosis has increased steadily over the past 30 years, with incidence of 2.1/100,000 in 1992 to 2.5/100,000 in 2018; however, the death rate has remained stable at approximately 0.5/100,000 women. The median age of vulvar cancer diagnosis is 68 years. The majority of vulvar cancers, approximately 75%, are squamous cell carcinomas (SCCs). However, numerous other histologic tumor types have been described. Many of these have clinical courses which differ from that of SCC. In this chapter, we will discuss several rare vulvar malignancies—HPV-independent vulvar cancers, verrucous carcinoma, aggressive angiomyxoma, and yolk sac tumors—and describe how their treatment may differ from the treatment of other, more common vulvar cancers. © 2023 Elsevier Inc. All rights reserved. |