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WHO Classification of Tumours
Paget disease, extramammary


Extramammary Paget disease (EMP) is a rare intraepidermal apocrine adenocarcinoma arising mainly in the female and male genital areas. A minority of cases involve axillae, eyelids and external auditory canals. The tumour may eventually invade the dermis and acquire metastatic potential. Primary EMP most likely originates from intraepidermal cells of apocrine gland ducts. Secondary EMP represents migration of an underlying internal malignancy to the epidermis.
EMP presents as a pruritic or burning, scaly, erythematous and sometimes ulcerated plaque. Histologically, the tumour is characterized by large, atypical neoplastic cells (Paget cells) featuring large nuclei with prominent nucleoli and abundant pale cytoplasm. They are scattered singly or in small clusters throughout the epidermis and are prone to track along skin appendages. The epidermis is often hyperkeratotic and acanthotic; the epidermal changes are accompanied by a dermal perivascular lymphohistiocytic infiltrate.
Extramammary Paget disease mainly affects patients >60 years of age. Genital EMP shows a predilection for women. Primary EMP has a recurrence rate of approximately 30%, but typically does not metastasize. Approximately 10% of patients develop invasive adenocarcinoma that may progress to metastatic disease. The prognosis for secondary EMP depends on the characteristics of the associated internal tumour

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LeBoit PE, Burg G, Weedon D, Sarasin A (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Skin Tumours
3rd Edition
IARC Press: Lyon 2005