Suburethral clear cell carcinoma of Müllerian origin: A case of preservation and discovery

Clear cell adenocarcinoma of the urethra is an exceedingly rare malignancy, with fewer than 0.003% of genitourinary cancers in females. We report a unique case of a 63-year-old woman diagnosed with a suburethral clear cell carcinoma of presumed Müllerian origin. The patient presented with intermittent urethral bleeding and a palpable mass. Imaging and cystoscopy confirmed a periurethral cystic lesion without urethral invasion. A transvaginal excision was performed, sparing the urethra and bladder. Histopathology revealed a papillary cystic tumor with clear cytoplasm, mitotic activity, and Müllerian immunohistochemical profile (CK7, Pax-8, HNF-1β positive). This case illustrates the feasibility of organ-preserving surgery in a tumor with typically aggressive behavior. The patient maintained full urinary function postoperatively, emphasizing the importance of individualized surgical planning in such rare malignancies. Long-term follow-up remains essential.