Vulva, Paget disease with invasion, CK7 stain

Paget disease of the vulva is an extramammary form of Paget disease of the nipple. It represents fewer than 2% of vulvar neoplasms, with an average patient age of 65 years. Invasion into the stroma can be seen in 10 to 20% of cases. The most common symptom is pruritus involving the labia, perineum, or anus. Clinically, it appears as an eczematoid, erythematous or ulcerated lesion. Histologically, Paget disease features large polyhedral cells with abundant pale cytoplasm, large nuclei, and small nucleoli, arranged either singly or in large clusters interspersed with normal squamous epithelial cells, frequently accompanied by epidermal hyperplasia, hyperkeratosis and parakeratosis, with involvement of the adnexal glands. It is positive for mucin, CK7, carcinoembryonic antigen (CEA), and Her-2-neu.

This slide shows CK7 stain. See Related Content for H&E stain.