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WHO Classification of Tumours
Paget disease, extramammary
Vulva


Definition

Extramammary Paget disease arising in the vulva is an uncommon, malignant intraepithelial neoplasm, most commonly of cutaneous origin, exhibiting glandular-like features and characterized by large neoplastic cells with abundant pale cytoplasm and large nuclei with prominent nucleoli (Paget cells).
Vulvar Paget disease usually affects postmenopausal women. 10-20% of cases have an invasive component or an underlying skin appendage adenocarcinoma

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Fanning J, Lambert HC, Hale TM, Morris PC, Schuerch C (1999)
Paget's disease of the vulva: prevalence of associated vulvar adenocarcinoma, invasive Paget's disease, and recurrence after surgical excision.
Am J Obstet Gynecol 180: 24-7




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Wilkinson EJ, Brown HM (2002)
Vulvar Paget disease of urothelial origin: a report of three cases and a proposed classification of vulvar Paget disease.
Hum Pathol 33: 549-54




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Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



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