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Paget's disease of the vulva

Paget's disease of the vulva

Question

Dear OncoLink "Ask The Experts,"
I need surgery for Paget's disease of the vulva. Can you tell me about my treatment options? 

Answer

Stephen C. Rubin, MD, Professor and Chief of the Division of Gynecologic Oncology, University of Pennsylvania Health System, responds:

Paget's disease of the vulva is an unusual kind of skin cancer that arises from glandular cells. Clinically, it can appear as red and white scaly plaques on the vulva. The tumor may frequently extend into what appears to be normal skin, making the appropriate extent of the surgical resection difficult to determine. For this reason, frozen section biopsies may be used to guide the surgery.

Approximately 15% of patients with vulvar Paget's disease will be found to have invasive Paget's disease or an underlying invasive adenocarcinoma of the vulva. There is also a reported association of vulvar Paget's with adenocarcinomas elsewhere in the body. The treatment for vulvar Paget's disease is surgical excision. In some cases with extensive involvement, skin grafting may be needed to close the resulting wound. Your treating physician can best advise you as to the necessary extent of the procedure.