原發性女性尿道腺癌之治療經驗及文獻回顧
陳威任1, 魏子鈞1,2, 郭俊逸1,2, 黃志賢1,2, 林登龍1,2, 陳光國1,2台北榮民總醫院 泌尿部1
國立陽明大學 醫學院 泌尿學科 及 書田泌尿科學研究中心2
Primary Female Urethral Adenocarcinoma – Report of 6 cases and Review of Literature
Wei-Jen Chen1, Tzu-Chun, Wei1,2, Junne-Yih Kuo1,2, William J.S. Huang1,2, Alex T.L. Lin1,2, Kuang-Kuo Chen1,2
Department of Urology1, Taipei Veterans General Hospital, Taiwan
Department of Urology, School of Medicine, and Shu-Tien Urological Institute2, National Yang Ming University, Taipei, Taiwan
Purpose: Female urethral carcinoma is rare and accounts for about 0.02% of all female cancers and less than 1% of cancers in the female genitourinary tract. It occurred mainly at proximal urethra and often presented with adjacent organ invasion and metastatic disease at diagnosis, resulting in poor prognosis. Literature was limited, with only 19 patients enrolled in the largest case series. We presented our treatment options and the outcome of female urethra adenocarcinoma in our hospital together with literature review.
Materials and Methods: From 2004 to 2016, six female patients with diagnosis of primary urethral adenocarcinoma in our hospital. The clinical data, treatment option, pathological feature and outcomes were retrospectively reviewed.
Results: The mean age was 63.3 years old (52-76). Five patients presented with difficult voiding initially, and the other one patient presented with post voiding urine dribbling while her adenocarcinoma was diagnosed within the urethral diverticulum. Two patients were diagnosed with local disease (group 1); the other 4 patients (group 2) were diagnosed with adjacent organ invasion (n=2) or initial metastatic disease (n=2). In group 2 patients, all received transurethral resection of tumor, and adjuvant chemotherapy. Two patients received further radiotherapy due to locally advanced disease (tumors confined in pelvis). For the 2 patients with initial metastatic disease out of pelvis, despite surgery and systemic chemotherapy, they were mortal 14 and 19 months after diagnosis. The 2 patients with initial locally advanced disease, who received transurethral resection of tumor, concurrent chemo-radiotherapy, survived 8 and 9 years respectively till now with disease under control. For the 2 patients with initial local disease, one patient received urethrectomy with suprapubic cystostomy for pT2 disease and has been followed without tumor recurrence for 4 years; the other one received wide tumor en bloc excision for pT1 disease, and has been followed for 6 months also without tumor recurrence.
Conclusions: Female urethral adenocarcinoma is an aggressive tumor, and the survival is poor if initial metastatic disease was diagnosed. For local disease or locally advanced disease which can be resected by anterior exenteration, surgical intervention is suggested according to our experience and other series, with or without chemotherapy or radiotherapy. For metastatic disease, whether chemotherapy or radiotherapy is beneficial is still controversial in literature due to rarity, however, in our series, aggressive tumor treatment by chemotherapy and radiotherapy seemed to be effective in patients with locally advanced disease confined within the pelvis.