Extramammary Paget's Disease of the Scrotal and Penile: A Case Report and Review of the Literature

Urol Int. 2024;108(3):264-271. doi: 10.1159/000536654. Epub 2024 Feb 17.

Abstract

Introduction: Extramammary Paget's disease of the scrotum and penis is a relatively rare cutaneous malignant tumor. At present, its pathogenesis, and clinical and pathological characteristics are not very clear. This is controversial regarding surgical margin width to decrease the high recurrence rate. This paper aimed to report the case and review the literature of extramammary Paget's disease of scrotum and penis.

Case presentation: We presented the case of a 74-year-old male patient with the patchy erythema and pruritus in the perineum who was admitted to our department. Biopsy of the large plaque revealed Paget disease. Under the condition of ensuring negative surgical margins by rapid frozen pathology, a wide local excision of the lesion, bilateral orchiectomy, and adnexectomy were performed on the patient. Pathology revealed that many scattered vacuolated Paget cells were observed in the epidermal layer, and the diagnosis was Paget's disease of the scrotum and penis. The 2 cm outside the skin lesion was used as the initial surgical margin, and free skin flap transplantation was used to repair the surgical wound. The patient recovered well and was discharged 1 week after surgery.

Conclusion: Currently, histopathologic biopsy is the most important diagnostic method for EMPD. Once confirmed, for patients eligible for surgical intervention, wide local excision of the lesion and rapid intraoperative frozen pathological examination should be performed as soon as possible. The skin flap transplantation is the first choice for the repair of large-scale wound after surgery.

Keywords: Clinicopathological characteristics; Extramammary Paget’s disease; Skin flap transplantation; Treatment methods.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Biopsy
  • Genital Neoplasms, Male / diagnosis
  • Genital Neoplasms, Male / pathology
  • Genital Neoplasms, Male / surgery
  • Humans
  • Male
  • Paget Disease, Extramammary* / diagnosis
  • Paget Disease, Extramammary* / pathology
  • Paget Disease, Extramammary* / surgery
  • Penile Neoplasms* / pathology
  • Penile Neoplasms* / surgery
  • Scrotum* / pathology
  • Scrotum* / surgery
  • Treatment Outcome

Grants and funding

This research was funded by the National Natural Science Foundation of China, Grant Numbers: 82260297 and 81960133, the Special Project of Yunnan Chronic Kidney Disease Clinical Medical Research Center, Grant Number: 202102AA100060, and the Yunnan University Women Pelvic Floor Dysfunction Research and Application of Science and Technology Innovation Team: K1322112.