Primary urethral carcinoma in female: An extremely rare case series at a single tertiary referral hospital and literature review.

INTRODUCTION AND IMPORTANCE: Primary urethral carcinoma (PUC) is exceedingly uncommon and accounts for 0.02 % of all female cancers and <1 % of female genitourinary tract malignancy. PUC in female usually presents late with a higher disease stage and, hence, has higher cancer-specific mortality. Due to its rarity, the current recommendation for the management of PUC is still unclear.

CASE PRESENTATION: This study reports two rare cases of 59-year-old and 65-year-old women with PUC, presenting with chief complaint of hematuria. Urethrocystoscopy and biopsy were performed. Pathology results revealed mucinous adenocarcinoma (AC) and urothelial carcinoma (UC) of the urethra, respectively. Radiological imaging was conducted for staging. Both were diagnosed with cT4N2M0. The first patient underwent anterior pelvic exenteration with bilateral ureterocutaneoustomy (UCS), while the second patient received cisplatin-based chemotherapy before the surgery. Radiological follow-up was planned 3 months after the surgery.

CLINICAL DISCUSSION: Both mucinous AC and UC are considered incredibly unusual subtypes, with no defined treatment guidelines. Anterior exenteration with or without neoadjuvant chemotherapy may be opted for advanced PUC affecting the proximal urethra and adjacent organs. Following the previous studies, in this case series, both patients (locally advanced) underwent anterior exenteration. Additionally, the UC subtype received multimodal treatment with neoadjuvant chemotherapy which was shown to improve overall survival.

CONCLUSION: In conclusion, PUC is extremely rare, and the choice of management remained various. Long-term follow-up for these patients is mandatory to improve understanding of this incredibly uncommon disease.