Extramammary Paget's disease with invasive adenocarcinoma of the penoscrotum: Case report and systematic review

Abstract Extramammary Paget's disease (EMPD) with an invasive adenocarcinoma of the penoscrotum is rare. In addition, high misdiagnosis and delayed diagnosis rates were also noted. In this article, we present a new case and a literature review to analyze the relationship between an internal malignancy and this disease. A 58-year-old man presented with a nonhealing pruritic and erythematous lesion on the right side of penile shaft and scrotum for 1 year. A provisional diagnosis of dermatitis was made by a dermatologist. A biopsy with immunohistochemical staining revealed Paget's disease with an invasive adenocarcinoma. Thorough preoperative systematic surveys showed no abnormality. The patient subsequently underwent wide local excision with negative margins and reconstruction with a split-thickness skin graft on the right side of the penile shaft and scrotum. Postoperatively, the wound healed well, and there was no obvious sign of local recurrence 5 months after the surgery. There is a high rate of misdiagnosis of EMPD. An internal malignancy related to a gastrointestinal cancer is rare in EMPD. Early diagnosis with a specific biopsy and adequate frozen section-guided wide excision contribute to a good prognosis for EMPD.