Management of Paget Disease of the Vulva
Extramammary Paget Disease (EMPD) of the vulva is a rare intra-epithelial adenocarcinoma of the vulvar skin presenting most commonly with pruritus. Unfortunately, diagnosis is often delayed as both patients and physicians dismiss initial symptoms or are misdiagnosed with benign conditions. There are two typical presentations: the vulvar skin can be red in appearance, occasionally with a violaceous hue (erythematous lesion) or this red epithelium can be partially covered with a thin white coating with a “cupcake frosting” appearance (eczematoid lesion).
The diagnosis is confirmed by punch biopsy with identification of vacuolated Paget cells. Immunohistochemical stains are necessary to differentiate between vulvar intraepithelial neoplasia, melanoma or other tumors. Standard treatment is wide local excision and multidisciplinary management with a gynecologic oncologist and plastic surgeon is often required. 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 skin graft may be required for extensive or recurrent disease.
Many clinicians perform frozen sections at the time of surgery to allow margin analysis, and extend the resection until all margins are negative. Because of its multifocal and multicentric nature, recurrence is common even when the surgical margins are clear. The disease has the ability to skip over normal appearing skin and can recur at sites distant to the original 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 which can impact their quality of life.
Although there are multiple case reports of alternative therapies for EMPD, none have been validated through randomized clinical trials. Carbon dioxide laser therapy has been used. Gauging the depth of treatment can be challenging and may result in repeat interventions. Photodynamic therapy uses a tumor sensitizing agent in combination with appropriate light wavelengths to destroy lesions. More recently, imiquimod, an immune modulator, has been successfully employed; it is a 5% cream applied to the affected region 3-4 times weekly for 6 months. Patients should be monitored for adverse events, including skin irritation, erosion, pain or ulceration, with dosing adjusted accordingly.
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 ranges from 14%-100% with 0-67% recurrences.
Older literature reports adenocarcinomas of the rectum, colon, bladder, breast and uterus associated with EMPD of the vulva. However, more recent studies have found a very low frequency of simultaneous lesions. Nonetheless, ACOG recommends further evaluation of the breast, genitourinary tract, and gastrointestinal tract in women with biopsy confirmed Paget Disease of the vulva. Paget lesions around the urethra and perianal areas require thorough evaluation for bladder or rectal tumors.
Eddey KA, Allan E, Murdoch JB, et al. Interventions for the treatment of Paget’s disease of the vulva. Cochrane Database Syst Rev. 2013 Oct 26;(10):CD009245.
American College of Obstetricians and Gynecologist; Clinical Updates in Women’s Healthcare: Common Dermatologic Conditions. Volume XVII, No. 1; January 2018:pp47-48. https://www.acog.org/clinical/journals-and-publications/clinical-updates/2018/01/common-dermatologic-conditions
Nasioudis D, Bhadra M, Ko EM. Extramammary Paget disease of the vulva: Management and prognosis. Gynecol Oncol. 2020 Apr;157(1):146-150. doi: 10.1016/j.ygyno.2019.11.009. Epub 2019 Nov 25. PMID: 31780234.
Initial Approval May 2010. Revised September 2015. Revised January 2018. Revised July 2019; Revised March 2021; Revised November 2022
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