Management of Paget Disease of the Vulva
5/1/2010 - Philip J. DiSaia, MD
Editor: Kenneth L. Noller, MD
Extramammary Paget Disease (EMPD) of the vulva is a rare intra-epithelial adenocarcinoma of the vulvar skin presenting mostly commonly with a complaint of pruritus. Unfortunately, diagnosis is often delayed as both patients and physicians dismiss the itch as due to local irritation from garments or other irritants. Inspection of the vulva reveals a redness of the skin with an occasional violaceous hue (erythematous lesion). At other times the red skin is partially covered with a thin white coating like “cupcake frosting” (eczematoid lesion).
The diagnosis is made by biopsy with identification of vacuolated Paget cells. Immunohistochemistry is necessary to exclude vulvar intra-epithelial neoplasia or melanoma. Standard treatment is wide local excision. Since invasion to structures below the dermis is rare, inguinal lymphadenectomy is not necessary unless the final pathology reports invasion. Very rarely, complete vulvectomy with appropriate skin graft may be required for extensive disease.
Many clinicians perform frozen section margin analysis at the time of surgery and extend the resection until all margins are negative. Unfortunately, because of its multifocal and multicentric nature, recurrence is common even when the margins are clear. The disease process appears to have the ability to skip over apparent normal skin and can recur at sites distant to the surgical site. For this reason, patients must be monitored closely and suspicious areas biopsied. In many patients, multiple local excisions are performed over a period of 10-20 years with the risk of vulvar disfigurement, impacting quality of life.
Although there are multiple case reports of alternative therapies for EMPD, none include randomized clinical trials. Carbon dioxide laser therapy has been used; however, gauging the depth of treatment can be challenging and may therefore result in repeat interventions. Photodynamic therapy uses a tumor sensitizing agent in combination with appropriate light wavelengths to destroy lesions. Photosensitivity can be problematic. More recently, Imiquimod, an immune modulator, has been successfully employed, typically used as a 5% cream 3-4 times weekly for 6 months. Patients should be monitored for adverse events, including skin irritation, erosion, pain or ulceration; dosing will have to be adjusted accordingly. A clinical trial evaluating the efficacy of Imiquimod is ongoing (www. clinicaltrials.gov, NCT02385188, accessed August 22, 2016).
Other therapies include radiotherapy and topical chemotherapeutic agents such as fluorouracil and bleomycin. Skin complications including pain, desquamation and ulceration are quite common. Regardless of treatment modality, reported treatment response range from 14%-100% and recurrence of 0-67%. The need for long term surveillance cannot be over-emphasized.
Older literature reports adenocarcinomas of the rectum, colon, bladder, breast and uterus associated with Paget Disease of the vulva. However, more recent studies have found a very low frequency of simultaneous lesions. Paget lesions around the urethra and perianal areas require careful evaluation for bladder or rectal tumors.
Eddey KA, Allan E, Murdoch JB, Cooper S, Bryant A. Interventions for the treatment of Paget’s disease of the vulva. Cochrane Database Syst. Rev. 10 (2013) CD009245
Machida H, Moeini A, Roman LD, Matsuo K. Effects of Imiquimod on vulvar Paget’s disease: A systematic review of literature. Gynecol Oncol 139 (2015) 165-171
Original Approval May 2010. Last Revised September 2015.
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