Extramammary Paget's disease (EMPD) is an adenocarcinoma of the apocrine glands with unknown exact prevalence and obscure etiology. It has been divided into primary EMPD and secondary EMPD, in which an internal malignancy is usually associated. Treatment for primary EMPD usually consists of wide lesion excision with negative margins. Multiple methods have been proposed to obtain free-margin status of the disease. These include visible border lesion excision, punch biopsies, and micrographic and frozen-section surgery, with different results but still high recurrence rates.

The investigators propose a method consisting of a staged contoured marginal excision using “en face” permanent pathologic analysis preceding the steps of central excision of the lesion and the final reconstruction of the surgical defect.

Advantages of this method include adequate margin control allowing final reconstruction and tissue preservation, while minimizing patient discomfort.

The staged contoured marginal and central excision technique offers a new alternative to the armamentarium for surgical oncologists for the management of EMPD in which margin control is imperative for control of recurrence rates.