Locally advanced female urethral adenocarcinoma of enteric origin: The role of adjuvant chemoradiation and brief review

Primary female urethral adenocarcinoma (FUA) is rare and has a poor prognosis. The common manifestations include urethrorrhagia, urinary frequency, dysuria, urethral obstructions, focal tenderness, and urinary tract infection. These symptoms are neither diagnostic nor pathognomonic; therefore, a delay in diagnosis and even a misdiagnosis is hardly uncommon. The histogenesis of FUAs may have derived from urethritis glandularis, Mullerian ducts, Skene’s glands, or mixed origins. Tumors of different embryologic origins displayed heterogeneous pathological morphology and immunohistochemistical phenotypes. Because of its rarity and the lack of large-scale studies, there is no current consensus on the optimal treatment of urethral adenocarcinomas. Here, we report two cases of locally advanced FUA of enteric origin. They manifested as slightest warning symptoms of urinary tract infection and stress urinary incontinence, respectively. One patient died of disease progression 2 months after curative operation. The other patient underwent surgery followed by adjuvant irinotecan-containing chemoradiation, and the effect was at least modest. Hence, we recommend adjuvant chemoradiation in locally advanced FUA. Individualizing cancer care of chemoregimens in accordance with the tumor origins may probably be beneficial in FUAs.

摘要原發性女性尿道腺癌為一罕見且預後不佳的惡性腫瘤。常見臨床表徵包含出血、頻尿、尿道阻塞症狀、局部疼痛或泌尿道感染。臨床因無特異表現，延誤診斷或誤診並非罕見。過去文獻證實原發性女性尿道腺癌病理型態具多樣性，因腫瘤起源自不同組織，而有不同細胞型態及免疫組織化學染色特徵。此外，原發性女性尿道腺癌屬罕見疾病且缺乏大型臨床試驗，目前對最佳治療方式缺乏共識。在此報告兩例女性尿道腺癌個案，病患均屬於局部性晚期病灶且起源自腸道，兩人分別以泌尿道感染及應力性失禁為初始表現。其中一人接受治癒性手術後2個月死於疾病進展，另一個病人則於術後接受輔助性放射線治療及含有喜樹鹼（irinotecan）的輔助性化學治療，其成效不俗。因而針對局部性晚期原發性女性尿道腺癌，建議病患術後接受輔助性化學及放射線治療，同時，依據腫瘤胚胎起源選擇個別不同的化療處方或許是未來可考量的方向。