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