EMPD: extramammary Paget disease MMS: Mohs micrographic surgery WLE: wide local excision INTRODUCTION Extramammary Paget disease (EMPD) is a rare intraepithelial adenocarcinoma of pluripotent keratinocyte stem cells, which typically occurs… Click to show full abstract
EMPD: extramammary Paget disease MMS: Mohs micrographic surgery WLE: wide local excision INTRODUCTION Extramammary Paget disease (EMPD) is a rare intraepithelial adenocarcinoma of pluripotent keratinocyte stem cells, which typically occurs in elderly patients. The current standard of care involves surgical treatment with either wide local excision (WLE) or Mohs micrographic surgery (MMS). For vulvar cases, radical resection, such as complete vulvectomy, may be performed. However, along with significant procedural morbidity, tissue margins are often positive following resection, occurring in as frequently as 97% of cases in some series. Even with reportedly negative pathologic margins, treatment of EMPD with WLE compared to that with MMS is associated with a 2.5-fold higher risk of recurrence in patients, which is likely due to incomplete margin evaluation and tumor removal. Modifications to the traditional MMS technique have been described to address lesions with large surface areas in order to combine the margin control of MMS with the need for resection and reconstruction under general anesthesia. A particular challenge arises in tumors located on the genitals surrounding orifices, where inner margin control is also necessary to achieve complete tumor clearance. Here, we report a case of vulvar EMPD treated with a novel modified dual inner and outer margin technique using MMS.
               
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