Surgical Experience With Genital and Perineal Extramammary Paget's Disease

Abstract

ObjectiveTo describe our surgical experience for the treatment and management of extramammary Paget's disease (EMPD). MethodsOur surgical approach involves excising a 2-cm margin of normal appearing skin around the EMPD-suspicious lesion. Prior to excision, the tissue is oriented and demarcated into predefined segments in coordination with a pathologist. Frozen sections are performed when necessary to guide additional excision. Xenograft or wet-to-dry dressings are applied depending on size and location of the wound while the specimen is expeditiously reviewed over the following 24-48 hours. If positive margins remain, further excision of the corresponding skin segment is performed. Delayed complex wound closure +/− split thickness skin grafting is performed once negative margins are confirmed. ResultsTen EMPD patients were referred to two academic centers between 2014 and 2018. Two patients had positive lymph nodes at diagnosis and underwent palliative surgery and died within 12 and 29 months. The remaining 8 patients underwent a median of 1 surgery (range 0-3) with referring providers before undergoing a median of 3 surgeries (range 2-5) at our institutions to achieve negative surgical margins and wound reconstruction (7 split thickness skin grafts, 1 secondary closure). At mean follow-up of 15 months, 1 patient recurred, required further excision, and remains disease free. ConclusionEMPD is a rare malignancy with poorly described treatment methodologies. Due to its multifocal distribution and asymmetric spread, obtaining negative margins can be challenging. Our systematic approach to obtaining wide margins and documenting excised skin has enabled us to achieve negative margins for this challenging malignancy.