A PAX-8-Positive Female Urethral Adenocarcinoma, Intestinal-Type: A Case Report with Diagnostic Challenges and a Review of the Literature

Authors

Mary M. Torrez, Department of Pathology, University of New Mexico, School of Medicine, Albuquerque, NM, USA
Frances M. Alba, Department of Surgery, Division of Urologic Surgery, University of New Mexico, School of Medicine, Albuquerque, NM, USA.
Jain Zhou, Department of Pathology, University of New Mexico, School of Medicine, Albuquerque, NM, USA.

Document Type

Article

Publication Date

1-1-2023

Abstract

Female urethral adenocarcinoma (FUA) is extremely rare. It is an aggressive malignancy, and clear cell and columnar/mucinous ("intestinal") represent the two primary histologic subtypes. Diagnosis is often delayed in patients because of their vague symptomatology; hence, they present with an advanced disease and a poor prognosis. The rarity of FUA brings challenges when determining treatment and management, and treatment guidelines for various stages are lacking. We report an intestinal-type FUA that developed from inflammation-related metaplasia in urethral diverticulum with positive paired box 8 (PAX-8) staining. In addition to intestinal-type FUA being extremely rare, this particular entity exhibiting PAX-8 positivity has not been previously described, to the author's best knowledge. The present report highlights the importance of clinical and radiological assessment as well as histomorphologic and immunophenotypic features for an accurate diagnosis of this rare and aggressive malignancy.

Recommended Citation

Torrez MM, Alba FM, Zhou J. A PAX-8-Positive Female Urethral Adenocarcinoma, Intestinal-Type: A Case Report with Diagnostic Challenges and a Review of the Literature. Case Rep Pathol. 2023 Feb 11;2023:8323821. doi: 10.1155/2023/8323821. PMID: 36820320; PMCID: PMC9938771.