Igor Snast1,2, Eran Sharon3,4, Ran Kaftory3, Yehonatan Noyman5,3, Meital Oren-Shabtai5,3, Moshe Lapidoth5,3, Emmilia Hodak5,3, Daniel Mimouni5,3, Sigal Mazor6, Assi Levi5,3. 1. Division of Dermatology, Rabin Medical Center-Beilinson Hospital, Petah Tikva, Israel, Snastigor@gmail.com. 2. Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel, Snastigor@gmail.com. 3. Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. 4. Department of Surgery, Breast Surgery Clinic, Rabin Medical Center, Petah Tikva, Israel. 5. Division of Dermatology, Rabin Medical Center-Beilinson Hospital, Petah Tikva, Israel. 6. Department of Community Dentistry, School of Dental Medicine, Hebrew University-Hadassah, Medical Center, Jerusalem, Israel.
Abstract
INTRODUCTION: Surgery is commonly regarded as the mainstay of treatment of extramammary Paget disease (EMPD); however, nonsurgical approaches have gained popularity in recent years. OBJECTIVES: To review the published evidence for the efficacy and safety of nonsurgical modes of therapy for EMPD. METHODS: A systematic review and meta-analysis of nonsurgical EMPD treatments was performed. The primary outcome was complete response (CR); secondary outcomes were clinical regression by ≥50%, adverse events, and recurrence rate. RESULTS: The systematic review included 43 observational studies (341 patients; 7 prospective cohort studies, 19 retrospective cohort studies, and 17 cases series) evaluating 5 treatment modalities. Imiquimod (13 studies, 110 patients) administered at variable doses ranging from daily to twice weekly for 2-56 weeks demonstrated CR of 54% (95% CI, 40-67%; I2 = 37%) and had a satisfactory safety profile. In 14 heterogeneous studies (122 patients) evaluating photodynamic therapy (PDT), only 36% (95% CI, 22-53%; I2 = 52%) of patients achieved CR. Radiotherapy (12 studies, 67 patients) showed CR of 97%, but was associated with local and systemic side effects. Ablative lasers and topical fluorouracil and calcipotriene lacked adequate evidence of efficacy. CONCLUSIONS: Imiquimod and radiotherapy are the most appropriate nonsurgical modalities for EMPD treatment given their good efficacy and safety profile. PDT has limited efficacy but may be appropriate in selected clinical settings.
INTRODUCTION: Surgery is commonly regarded as the mainstay of treatment of extramammary Paget disease (EMPD); however, nonsurgical approaches have gained popularity in recent years. OBJECTIVES: To review the published evidence for the efficacy and safety of nonsurgical modes of therapy for EMPD. METHODS: A systematic review and meta-analysis of nonsurgical EMPD treatments was performed. The primary outcome was complete response (CR); secondary outcomes were clinical regression by ≥50%, adverse events, and recurrence rate. RESULTS: The systematic review included 43 observational studies (341 patients; 7 prospective cohort studies, 19 retrospective cohort studies, and 17 cases series) evaluating 5 treatment modalities. Imiquimod (13 studies, 110 patients) administered at variable doses ranging from daily to twice weekly for 2-56 weeks demonstrated CR of 54% (95% CI, 40-67%; I2 = 37%) and had a satisfactory safety profile. In 14 heterogeneous studies (122 patients) evaluating photodynamic therapy (PDT), only 36% (95% CI, 22-53%; I2 = 52%) of patients achieved CR. Radiotherapy (12 studies, 67 patients) showed CR of 97%, but was associated with local and systemic side effects. Ablative lasers and topical fluorouracil and calcipotriene lacked adequate evidence of efficacy. CONCLUSIONS:Imiquimod and radiotherapy are the most appropriate nonsurgical modalities for EMPD treatment given their good efficacy and safety profile. PDT has limited efficacy but may be appropriate in selected clinical settings.