Extramammary Paget disease. Part I. epidemiology, pathogenesis, clinical features, and diagnosis Review


Authors: Shah, R. R.; Shah, K.; Wilson, B. N.; Tchack, M.; Busam, K. J.; Moy, A.; Leitao, M. M.; Cordova, M.; Neumann, N. M.; Smogorzewski, J.; Nguyen, K. A.; Hosein, S.; Dafinone, M.; Schwartz, R. A.; Rossi, A.
Review Title: Extramammary Paget disease. Part I. epidemiology, pathogenesis, clinical features, and diagnosis
Abstract: Extramammary Paget disease (EMPD) is a rare skin cancer of apocrine-rich skin that mimics common inflammatory and infectious dermatoses, leading to delays in diagnosis and increased patient morbidity. Better clinical recognition of this entity, multidisciplinary patient assessment, and deeper understanding of the underlying pathophysiology are essential to improve patient care and disease outcomes. It is important to distinguish primary intraepithelial/micro-invasive EMPD from invasive EMPD or cases with adenocarcinoma arising within EMPD. This 2-part continuing medical education series provides a complete picture of EMPD. Part 1 of this continuing medical education series reviews the epidemiology, oncogenesis, clinical and histopathologic presentation, workup, and prognosis of this rare cancer. © 2024 American Academy of Dermatology, Inc.
Keywords: immunohistochemistry; clinical article; human tissue; aged; middle aged; clinical feature; histopathology; pathogenesis; review; pathophysiology; sentinel lymph node biopsy; skin neoplasms; diagnosis, differential; differential diagnosis; cohort analysis; patient assessment; immunofluorescence; pathology; carcinogenesis; skin tumor; drug combination; diagnosis; epidemiology; adverse drug reaction; immunohistochemical markers; etiology; sentinel node biopsy; paget skin disease; humans; prognosis; human; male; female; extramammary paget disease; paget disease, extramammary; neoplastic cell transformation; paget cells; primary empd; secondary empd
Journal Title: Journal of the American Academy of Dermatology
Volume: 91
Issue: 3
ISSN: 0190-9622
Publisher: Mosby Elsevier  
Date Published: 2024-09-01
Start Page: 409
End Page: 418
Language: English
DOI: 10.1016/j.jaad.2023.07.1051
PUBMED: 38704032
PROVIDER: scopus
DOI/URL:
Notes: The MSK Cancer Center Support Grant (P30 CA008748) is acknowledge in the PDF -- Corresponding authors is MSK author: Anthony Rossi -- Source: Scopus
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MSK Authors
  1. Mario Leitao
    575 Leitao
  2. Klaus J Busam
    688 Busam
  3. Anthony Rossi
    233 Rossi
  4. Andrea Primiani Moy
    32 Moy
  5. Sharif Hosein
    10 Hosein