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D efinition —Invasive epithelial neoplasm composed of keratinocytes involving the vulva. Human papillomavirus (HPV) negative.
Typically affects women in their seventh and eighth decades and is usually found associated with inflammatory dermatosis (differentiated vulvar intraepithelial neoplasia [VIN]).
Some patients present with no history of vulvar neoplasia.
Not associated with HPV and not associated with squamous neoplasms of the cervix.
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