Vulvar pagetoid urothelial intraepithelial neoplasia: a case report

Abstract

Background

Pagetoid urothelial intraepithelial neoplasia (PUIN) is a form of secondary Extramammary Paget Disease (EMPD). It is a rare malignant condition seen on the female genitalia synchronous or metachronous with bladder cancer (BC).

Case presentation

A 66-year-old female presented with PUIN at the labia minora 2 years after an open anterior pelvic exenteration with ileal conduit urinary diversion for carcinoma in situ (CIS) of the bladder. PUIN 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 EMPD was identified by the expression tumor protein 63 (p63), cytokeratin 7, and cytokeratin 20 (CK20).

Conclusions

PUIN 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.

Keywords:

• Pagetoid urothelial intraepithelial neoplasia
• extramammary Paget
• bladder cancer
• carcinoma in situ
• urothelial cell carcinoma

Ethics approval

Approved by Ethics Committee of AZ Damiaan Hospital Belgium.

Informed consent

Written informed consent for publication of their clinical details and/or clinical images was obtained from the patient. A copy of the consent form is available for review by Editor of this journal.

Author contributions

Writing by SB and EL, surgery by DP, NL and PT, review and editing by CVP, KD, JD and PM. All authors read and approved the final manuscript.

Disclosure statement

The authors report no conflict of interest.

Data availability statement

All data generated during this case report are included in this published article.