Recurrent primary scrotal extramammary Paget disease: a case report and literature review

Abstract

Extramammary Paget disease (EMPD) is a rare malignant neoplasm arising in apocrine gland-rich skin, which may be classified as either of primary or secondary origin. Management of this condition is predominantly surgical, and is often characterised by lengthy diagnostic delays. Complete surgical excision is challenging, and local recurrence is common. Herein, we discuss a subtle presentation of recurrent scrotal EMPD in a 77-year-old male and review the available literature. Although relatively rare, the indistinct nature of this pathology merits special attention from treating surgeons, who are frequently responsible for initial management and follow-up. The risk of distant metastasis and concomitant prognostic implications necessitate a high clinical index of suspicion, and low threshold for definitive biopsy in similar cases.