Management of secondary Paget's disease of the vulva associated with transitional cell carcinoma

J Cancer Res Clin Oncol. 2022 Jul;148(7):1697-1702. doi: 10.1007/s00432-022-04007-z. Epub 2022 Apr 17.


Purpose: Secondary extramammary Paget's disease (EMPD) related to urothelial carcinoma is rare, with some cases presenting synchronously with either a primary neoplasm or recurrence of a neoplasm and other cases presenting up to 13 years prior to the detection of urothelial carcinoma. In this report, we will review the presentation, diagnosis, pathophysiology, management, and literature review of cases of secondary EPMD associated with urothelial carcinoma.

Methods: We reviewed the English literature for all cases of secondary EMPD presenting synchronously with or in patients with a history of urothelial carcinoma, as well as treatment data for secondary vulvar Paget's.

Results: We identified 16 case reports and case series with a total of 20 cases of vulvar EMPD associated with urothelial carcinoma. Twelve cases presented asynchronously and 8 had EMPD preceding the diagnosis of the underlying neoplasm. There is a paucity in the literature regarding management and surgical resection is a common treatment strategy; however, nonsurgical interventions may also be effective.

Conclusion: There is a paucity in the literature regarding management of secondary EPMD of urothelial origin, but consideration of radiation and systemic chemotherapy may be a reasonable treatment approach.

Keywords: Immunohistochemistry; Secondary vulvar Paget’s disease; Transitional cell carcinoma; Urothelial carcinoma; Vulvar Paget’s disease.

Publication types

  • Review

MeSH terms

  • Carcinoma, Transitional Cell* / diagnosis
  • Carcinoma, Transitional Cell* / therapy
  • Female
  • Humans
  • Paget Disease, Extramammary* / diagnosis
  • Paget Disease, Extramammary* / therapy
  • Urinary Bladder Neoplasms* / diagnosis
  • Urinary Bladder Neoplasms* / therapy
  • Vulva / pathology
  • Vulvar Neoplasms* / diagnosis
  • Vulvar Neoplasms* / pathology
  • Vulvar Neoplasms* / therapy