Nonsurgical Treatments for Extramammary Paget Disease: A Systematic Review and Meta-Analysis

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%; I 2 = 37%) and had a satisfactory safety profile. In 14 heterogeneous studies (122 patients) evaluating photodynamic therapy (PDT), only 36% (95% CI, 22-53%; I 2 = 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.

Extramammary Paget’s Disease of the Vulva: Report of Two Cases

“…Most retrospective studies, including a large meta-analysis by Snast et al [ 16 ], indicate that EMPD seems to affect women more often, probably as a result of the condition’s predominance in the vulvar area [ 4 , 6 , 8 , 10 , 12 ]. However, in one retrospective study of 76 Japanese patients, Hatta et al have found a significantly higher prevalence in men; in this particular study, 72% were male and 28% female [ 14 ].…”

“…Non-surgical techniques have also been developed or used in vulvar EMPD, most of them as complementary treatments to the surgical ones. Snast et al, in their recent meta-analysis for non-surgical treatments for EMPD, have reviewed the efficiency of the options available in the literature, specifically radiotherapy, photodynamic therapy (PDT), laser therapy, immune response modifiers, and locally applied creams [ 16 ]. Radiation is mostly used in elderly patients where comorbidities do not allow surgical intervention, in combination with surgical treatments as adjuvant radiation [ 13 ], or in difficult anatomic areas where complete excision of the lesion is not feasible [ 16 , 20 , 21 ].…”

“…Snast et al, in their recent meta-analysis for non-surgical treatments for EMPD, have reviewed the efficiency of the options available in the literature, specifically radiotherapy, photodynamic therapy (PDT), laser therapy, immune response modifiers, and locally applied creams [ 16 ]. Radiation is mostly used in elderly patients where comorbidities do not allow surgical intervention, in combination with surgical treatments as adjuvant radiation [ 13 ], or in difficult anatomic areas where complete excision of the lesion is not feasible [ 16 , 20 , 21 ]. Laser ablation (CO 2 , holmium, argon dye) has been used on several occasions; nevertheless, high local recurrence rates have been reported and patients complain of severe pain [ 13 , 16 , 20 , 21 ].…”

Definitive Radiotherapy for Penoscrotal Extramammary Paget’s Disease: A Case Report with Long-Term Follow-Up

“…[2][3][4][5] In a recent review that included 12 studies (4 cohort studies and 8 cases series) with a total of 67 patients, the treatment results of RT were evaluated. 2 Although the interpretation of this analysis may be difficult because the radiation fields, doses, and schedules were not standardized between patients; the complete response rate was 97%, which is comparable with the reported rate for surgery and higher than that for other nonsurgical modalities. 2 These results show that RT can be an ideal therapeutic modality for treating extensive lesions such as that in our patient.…”

“…Nonsurgical interventions have also been investigated currently, with varied results. 2 Radiotherapy (RT) has been suggested as a less invasive and potentially curative treatment, with a lower risk of adverse effects, [2][3][4][5] but the existing published evidence has several limitations. Most articles report small-scale retrospective studies, referring to patients treated with large heterogeneities in terms of RT dose and technique.…”