Management of peno-scrotal extramammary Paget's disease: surgical treatment spectrum and our initial experience

Abstract

Objective: Extramammary Paget’s disease (EMPD) of the male external genitalia is extremely uncommon. We aimed to investigate consecutive patients with penoscrotal EMPD in the contemporary era, treatment involved and outcomes. Patients & Methods: Prospective patients with histopathologically confirmed peno-scrotal EMPD were enrolled between September 2017 to June 2019. Clinical presentation, pathology, oncological outcomes, and spectrum of surgical treatment implemented were analysed. Results: 5 patients were eligible. All had unremarkable cystoscopy and colonoscopy. 4 patients treated with frozen-section mapping-guided surgery. 2 patients had wide local excision (WLE) with local skin-flap reconstruction. 2 patients required extensive WLE with local skin flap and partial-thickness skin graft reconstruction. 1 patient with history of EMPD treated 16 years ago had distal urethral recurrence, underwent mini-Johansson’s procedure and subsequent topical imiquimod therapy. All patients had negative surgical margins. 1 patient had stromal invasion and subsequent dynamic inguinal sentinel lymph node confirmed occult nodal metastasis. All other patients remain disease-free on follow-up Conclusion: Frozen-section mapping-guided surgical resection for peno-scrotal EMPD is crucial to achieve tumour-free margins. A wide spectrum of reconstructive techniques is required for personalized local EMPD treatment. Appropriate nodal assessment is essential for patients with invasive EMPD.