Extramammary Paget's Disease of genitourinary origin. Issue 2 (March 2022)

• Record Type: Journal Article
• Authors: Narayan, Vikram M.; Master, Viraj A.
• Is Part Of: Current opinion in urology. Volume 32:Issue 2(2022)
• Publication Date: 2022-03
• Subjects: Extramammary Paget's Disease -- extramammary Paget's malignancy -- genitourinary Extramammary Paget's Disease -- rare genitourinary tumors; Urology -- Periodicals; Review Literature -- Bibliography; Review Literature -- Periodicals; Male Urogenital Diseases -- Bibliography; Male Urogenital Diseases -- Periodicals; Female Urogenital Diseases -- Bibliography; Female Urogenital Diseases -- Periodicals; Urologic Diseases -- Bibliography; Urologic Diseases -- Periodicals; Periodicals; 616.6005
• DOI: 10.1097/MOU.0000000000000948
• Languages: English
• ISSNs: 1473-6586

Abstract

Abstract : Purpose of review: Extramammary Paget's Disease (EMPD) is an uncommon intra-epithelial malignancy, affecting primarily apocrine gland-bearing skin. EMPD is often considered an orphan diagnosis given its rarity. This review provides a contemporary overview of EMPD management. Recent findings: The mainstay of EMPD treatment centers around a high index of suspicion to allow for an early and accurate diagnosis, wide local or Mohs micrographic surgical excision with care paid toward the margin status, and thoughtful consideration for lymphadenectomy in patients with clinically positive regional disease. There is currently no consensus regarding adjuvant therapies or systemic therapies although with ongoing improvements in tumor biology and genomics, including molecular pathways and alterations specific to EMPD, targeted or combinatorial therapies may be on the horizon. Summary: Clinicians caring for patients with EMPD should seek consultation from or if feasible, consider referral to high-volume, experienced centers with patients counseled and provided with frequent and close follow-up for disease recurrence or progression. Collaboration with groups such as the Global Society for Rare Genitourinary Tumors, and especially patient groups will be vital to designing trials and collaborative databases.