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Mammary and extramammary Paget's disease

J Lloyd¹ and A M Flanagan²

¹ Department of Histopathology, Northwick Park and St Mark's NHS Trust, Watford Road, Harrow, Middlesex HA1 3UJ, UK
² Imperial College School of Medicine (St Mary's Campus), Norfolk Place, London W2 1PG, UK

Correspondence to:
Dr Lloyd 8spook8{at}excite.com

Mammary and extramammary Paget's disease are uncommon intraepithelial adenocarcinomas. Both conditions have similar clinical features, which mimic inflammatory and infective diseases. Histological diagnostic confusion can arise between Paget's disease and other neoplastic conditions affecting the skin, with the most common differential diagnoses being malignant melanoma and atypical squamous disease. The glandular differentiation of both mammary Paget's disease and extramammary Paget's disease is indicated by morphological appearances, the presence of intracellular mucin in many cases, and positive immunohistochemical staining for glandular cytokeratins, epithelial membrane antigen, and carcinoembryonic antigen. This article provides an overview of mammary and extramammary Paget's disease and discusses recent evidence regarding the cell of origin. The concepts of primary and secondary Paget's disease are presented and the differential diagnosis is discussed with reference to immunohistochemical markers that might be of diagnostic value.

Key Words: mammary Paget's disease • extramammary Paget's disease

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This article has been cited by other articles:

• Chanjuan Shi, , Argani, P. (2009). Synchronous Primary Perianal Paget's Disease and Rectal Adenocarcinoma: Report of a Hitherto Undescribed Phenomenon. INT J SURG PATHOL 17: 42-45 [Abstract]  
• Lester, T., Wang, J., Bourne, P., Yang, Q., Fu, L., Tang, P. (2009). Different Panels of Markers Should Be Used to Predict Mammary Paget's Disease Associated with In Situ or Invasive Ductal Carcinoma of the Breast. Annals of Clinical & Laboratory Science 39: 17-24 [Abstract] [Full Text]  
• Bhatia, P, Ahuja, A, Dey, P, Suri, V (2007). Vulval intraepithelial neoplasia with extra mammary pagets disease: a rare association. J. Clin. Pathol. 60: 110-112 [Full Text]