Imiquimod therapy for extramammary Paget's disease of the vulva: A viable non-surgical alternative

Abstract

Extramammary Paget's disease (EMPD) is a rare intraepidermal adenocarcinoma that can affect the vulval skin. Surgical excision is the gold-standard treatment, however, recurrence rates are high and extensive excisions can produce long-lasting cosmetic and functional defects. We describe one of the largest case series to-date (n = 6) on the use of topical 5% imiquimod cream as a novel treatment option and discuss our experiences. With the addition of our six cases to the literature, there are now 29 documented cases of vulval EMPD treated with 5% imiquimod cream. Of these, 50% of primary disease cases and 73% of recurrent primary disease cases have achieved clinical resolution with 5% imiquimod therapy alone. These findings suggest that imiquimod provides a viable alternative to surgical excision for vulval EMPD. However, we acknowledge that this is a simple retrospective analysis and that treatment scheduling and follow-up needs investigation in a trial setting.

Paget's Disease of the Vulva Treated with Imiquimod: Case Report and Systematic Review of the Literature

“…Severe side effects as described in the literature did not occur among women with EPDV. The only systemic side effects noted among this patient collective Local reaction (1) 11/6/0; premature discontinuation (2); stable disease (2) Sanderson et al [16] 2013 PRI (5), REC (1) Retrospective cohort 6 2, 2, 2, 4, 4, 4…”

“…In the next step, 18 articles were further excluded for the following reasons: cases were not EPDV but Paget's disease in other locations (n = 12); patients already reported in a previous publication (n = 1); narrative review without reporting new cases (n = 3); extramammary Paget's disease without reporting the exact location (n = 1); EPDV, but not treated with imiquimod (n = 1). Finally, 20 articles [15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33][34] and our own case containing data of 70 cases of EPDV treated with imiquimodincluding our own case -were extracted and analyzed. From these studies, patients numbers, efficacy rates in terms of CR, PR, stable disease (where available), and progressive disease, the rate of treatment discontinuation, side effects, and length of follow-up were extracted.…”

“…In another retrospective case series, Sanderson et al [16] described 6 women with primary and recurrent EPDV treated with imiquimod. Adding 23 cases previously published in the literature, the authors cite clinical resolution rates of 50% for primary disease and 73% for recurrent disease based on 29 documented EPDV cases treated with imiquimod.…”

Extramammary Paget Disease of the Vulva - Case Report

“…Extramammary Paget disease (EPD) is a rare neoplasm that occurs in areas containing apocrine glands and affects the epidermis and, occasionally, the derm. [1][2][3] The most commonly affected site is the vulva, but it may also affect the axilla, perineum and perianal region. 1,2 Vulvar Paget disease is more common from the 5th decade of life on, 2 with an average age at diagnosis from 61 to 69 years, depending on the study.…”

The case of an extensive primary extramammary Paget's disease diagnosis and treatment

“…Hence, alternative local treatments have been evaluated. Imiquimod 5% topical cream [9][10][11], photodynamic therapy [12,13] and radiation therapy [14] are the most frequently applied alternative treatments.…”