楊哲學 林益聖 翁瑋駿 黃立華 呂謹亨 歐宴泉 許兆畬 童敏哲

台中童綜合醫院 外科部 泌尿科

Che-Hsueh Yang, Yi-Sheng Lin, Wei-Chun Weng, Li-Hua Huang, Chin-Heng Lu, Yen-Chuan Ou, Chao-Yu Hsu, Min-Che Tung

Division of Urology, Department of SurgeryS

Tungs' Taichung MetroHarbor Hospital, Taichung, Taiwan

 

Case: A woman, aged 75 and with end-stage renal disease under hemodialysis, visited us first time for left hydronephrosis, accompanied with urinary tract infection. After ultrasound and lab assessment, computed tomography was done, and then tumors at bladder, left ureteral orifice, and left renal calyx were found, causing obstruction. Several enlarged lymph nodes were found at para-aortic site. Subsequently, cystoscopy and left ureteroscopy biopsy were suggested. Prior to that, left percutaneous nephrostomy was indicated to control her infection and release the hydronephrosis and obstruction condition. One week after, cystoscopy and left ureteroscopy biopsy were operated, then indwelled with a ureteral stent. Intra-operatively, we observed total 4 visible tumors, one at urinary bladder, one at left lower third ureter, one at left upper third ureter, and the other at left renal pelvic. All sessile in appearing ranged from 2 cm in width, minimally measured at left upper third ureter, to 10 cm in width, maximally measured at left renal pelvic. Pathology report 2 weeks after showed invasive lymphoepithelioma-like carcinoma (LELC). Whole body bone scan and positron emission tomography were arranged and no signs of distant organs and bone metastasis. Hence, robotic-assisted left nephroureterectomy and bladder cuff excision with partial cystectomy were proposed to her and scheduled to be performed. Total 4 locations in specimen, a 10.5 x 9.8 cm tumor at left renal pelvic, a 2.5 x 1.4 cm tumor at left upper third ureter, a 8.9 x 3.5 cm tumor at lower third ureter, and one 2.1 x 1.1 cm tumor at bladder were dug out. All of them presented with cell type of invasive LELC. Except for pT3 at left renal pelvic, the rest 3 all were pT2, and no recurrence was seen half a year after surgery.

Discussion: Cancer histology of urothelial origin nearly makes up of 90% in majority along the upper urinary tract, also the lower urinary tract. lymphoepithelioma is most seen at upper respiratory tract, said to be linked with EBV infection. Typically speaking, in Asia it presents as an undifferentiated form carcinoma at nasopharynx. In this way, carcinoma histology features alike but appears at the sites other than nasopharynx will be termed as LELC. First published in 1991 by Zukerberg et al. and, for its rare occurrence at urinary tract, for example nearly 1% in bladder, no guidelines can be applied to treatment. LELC can be found 2 types, mixed with other cancer histology or pure. Terminology is close to lymphoepithelioma at nasopharynx, LELC acts totally different behavior under FISH test against it though. More chromosomal abnormalities resembling to urothelial carcinoma and frequent p53 positivity, but neither hybridization of HPV nor EBV immunostaining characteristics can be examined. Most opinions now deem LELC as a high grade invasive urothelial carcinoma, supported by FISH and other immuohistoological studies. Survival analysis, done by Antonio et al. in 2017, showed no differences with the conventional urothelial carcinoma at both upper and lower urinary tracts, and pathological T stage is the parameter to prognosis. Under this basis, it gives us hints to do treatments. Yang et al. in 2017 reviewed 140 cases of bladder LELC, concluding multi-modality treatment will lead to more disease-free survival and less mortality rates than TURBT alone. However, when TURBT is added with intravesical chemotherapy, treatment described by Sean et al. in 2011, it will decrease the likelihood of recurrence. Pure type LELC, at current studies on this topic in majority, is concluded to be with better outcome in terms of recurrence, mortality, disease-free survival and overall survival. There are studies telling high expression of PD-1 and PD-L1 in lung LELC, thus usage of immunotherapies like nivolumab is hypothesized, but still lack the related information in urinary tract LELC. The case we presented in this abstract is a perfect case having both upper and lower urinary tracts involved. Under the evidence for now, it is reasonable to treat it like the ways we do in urinary tract urothelial carcinoma. That is nephroureterectomy with bladder cuff excision to upper urinary tract LELC, TURBT with intravesical chemotherapy to non-muscle invasive LELC, and radical or partial cystectomy with neoadjuvant or adjuvant chemotherapy to muscle invasive LELC. Chances still exist in distant metastasis like urothelial carcinoma especially those with muscle invasive type. Follow-up strategies better adhere to that of urothelial carcinoma at urinary tract as well.