Abstract

Vulvar squamous cell carcinoma is a rare and heterogeneous disease. Young women who develop vulvar squamous cell carcinoma tend to develop non-keratinizing histological types (warty and basaloid squamous cell carcinomas) in the setting of warty and/or basaloid-type usual vulvar intraepithelial neoplasia (uVIN); these tumors are commonly human papillomavirus (HPV) related. Older women who develop vulvar squamous cell carcinoma tend to develop keratinizing squamous cell carcinoma often adjacent to differentiated-type VIN; these tumors are not HPV related, rather they frequently arise in the setting of lichen sclerosus (LS). Keratinizing squamous cell carcinoma is the most commonly encountered histological type, but rare histological variants of squamous cell are sometimes identified such as verrucous carcinoma, keratoacanthoma-like squamous cell carcinoma, sarcomatoid carcinoma, and squamous cell carcinoma with tumor giant cells. Regardless of the tumor histology and etiology, surgery is the mainstay of staging and treatment for patients with resectable disease.

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