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Vulvar Pagetoid Urothelial Intraepithelial Neoplasia: a case report.

Background Pagetoid urothelial intraepithelial neoplasia is a form of secondary extramammary Paget disease. It is a rare malignant condition seen on the female genitalia synchronous or metachronous with bladder cancer.Case presentation A 66-year-old female presented with pagetoid urothelial intraepithelial neoplasia at the labia minora two years after an open anterior pelvic exenteration with ileal conduit urinary diversion for carcinoma in situ of the bladder. Pagetoid urothelial intraepithelial neoplasia of the vulva and vagina was confirmed by a punch biopsy and the patient underwent a radical vaginectomy with urethrectomy and inguinal sentinel node procedure. Immunohistochemically extramammary Paget disease was identified by the expression tumor protein 63, cytokeratin 7 and cytokeratin 20.Conclusion Pagetoid urothelial intraepithelial neoplasia is a rare but distinct clinical entity as a form of secondary EMPD which can be differentiated from primary EMPD based on medical history, histology and immunohistochemistry.