Treatment Outcomes of Adjunct and Stand-alone Photodynamic Therapy in Patients With Extramammary Paget Disease: A Literature Review

BACKGROUND Extramammary Paget disease (EMPD) is a rare malignant neoplasm arising from apocrine gland–bearing skin. The surgical management of EMPD is often coupled with noninvasive techniques including cryotherapy, ablative lasers, topical chemotherapies, and photodynamic therapy (PDT). The specificity and preservation of tissue that PDT with photosensitizers 5-aminolevulinic acid or 5-methyl aminolevulinate allows makes it a potential treatment of EMPD. METHODS The authors present a review of 13 studies, from 2002 to 2019, examining the reported efficacy of PDT alone and adjunctive PDT in EMPD treatment. RESULTS In the 52 patients with 56 lesions who received stand-alone PDT, 20 lesions (35.7%, n = 20/56) experienced complete resolution, 31 lesions (55.4%, n = 31/56) experienced partial resolution, 5 lesions (8.9%, n = 5/56) failed to demonstrate response to treatment, and 23 lesions (41.1%, n = 23/56) had recurrence. In the 56 patients with 66 lesions that received adjunctive PDT paired with surgery ( n = 55/66), imiquimod ( n = 4/66), holmium laser and surgery ( n = 1/66), Mohs surgery ( n = 2/66), and combined surgery, imiquimod, and 5-fluorouracil ( n = 1/66), 34 lesions (51.5%) experienced complete resolution, 27 lesions (40.9%) experienced partial resolution, 5 lesions (7.6%) failed to demonstrate any response to treatment, and 16 lesions (24.2%) had EMPD recurrence. CONCLUSION Further studies with larger sample size are needed to consolidate these findings and inform clinical decisions.