Mammary and extramammary Paget's disease

• Published in: Journal of clinical pathology Vol. 53; no. 10; pp. 742 - 749
• Main Authors: Lloyd, J, Flanagan, A M
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd and Association of Clinical Pathologists 01.10.2000; BMJ; BMJ Publishing Group LTD
• Subjects: Biological and medical sciences; Breast Neoplasms - pathology; Breasts; Dermatology; Diagnosis, Differential; Disease; extramammary Paget's disease; Female; Gynecology. Andrology. Obstetrics; Humans; Leader; Mammary gland diseases; mammary Paget's disease; Medical sciences; Melanoma; Morphology; Mutation; Paget Disease, Extramammary - pathology; Paget's Disease, Mammary - pathology; Pathogenesis; Skin cancer; Skin involvement in other diseases. Miscellaneous. General aspects; Tumors; Vulvar Neoplasms - pathology; Human; Immunohistochemistry; Skin disease; Anorectal; Vulva; Review; Male genital system; Differential diagnostic; Female genital diseases; Paget disease of breast; Pathology; Mammary gland diseases; Extramammary Paget disease; Axilla; Cytogenetics; Digestive diseases; Vulvar diseases; Clinical investigation; Diagnosis; Male genital diseases; Anorectal disease
• ISSN: 0021-9746; 1472-4146
• DOI: 10.1136/jcp.53.10.742

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.