Abstract
Vulvar squamous cell carcinoma (SCC) is an etiologically heterogeneous entity evolving on the basis of two separate pathways. Most tumors originate from gene mutations (especially tumor suppressor gene TP53) in the environment of chronic vulvar dermatoses independently on human papillomavirus (HPV) infection.
Minority of cases belongs to the spectrum of multicentric lower genital tract neoplasia which is causally linked to sexually transmitted high-risk HPV infection. Each etiopathogenetic sequence is defined by a histologically distinct precancerous lesion - HPV positive high-grade squamous intraepithelial lesion (HSIL), formerly known as vulvar intraepithelial neoplasia, usual type (uVIN), and HPV negative vulvar intraepithelial neoplasia, differentiated type (dVIN) which behave in a more aggressive manner and may be clinically as well as histologically inconspicuous.
HPV associated tumors represented by warty and basaloid subtypes of vulvar SCC tend to occur in a significantly younger age and have a better prognosis contrary to HPV negative group composed mainly of well differentiated keratinizing SCC which affects older postmenopausal women. The role of pathologist remains in the typing, grading and staging of the tumor, including evaluating of pathologic risk factors, but may be potentially expanded by specialized investigations for the indication of individualized tailored therapy.