原發性輸尿管小細胞癌合併膀胱尿路上皮癌：罕見病例報告

林奇柏¹、陳昭安²、陳俊吉¹、石宏仁¹、林介山¹、張進寶¹、王百孚¹、
嚴孟意¹、黃勝賢¹、江恆杰¹、張建祥¹、陳柏華¹、陳一中¹、潘岳¹

¹彰化基督教醫院泌尿外科; ²昭安泌尿科診所

Primary small cell carcinoma of the ureter and concurrent urothelial carcinoma of the bladder: a rare case report

Chi-Bo Lin¹, Chao-An Chen², Chun-Chi Chen¹, Hung-Jen Shih¹, Jesun Lin¹, Chang-Pao Chang¹, Bai-Fu Wang¹, Meng-Yi Yan¹, Sheng-Hsien Huang¹, Herng-Jye Jiang¹, Jian-Xiang Zhang¹, Pao-Hwa Chen¹,
Yi-Zhong Chen¹, Pan Yueh¹

¹Division of Urology, Department of Surgery, Changhua Christian Hospital; ²Morning urology clinic

Primary small cell carcinoma (SCC) of the urinary tract is a rare form of cancer, making up less than 0.5% of urinary tract tumors. It is primarily localized in the bladder and prostate, with extremely rare occurrences in the renal pelvis or ureter. Smoking exposure is a significant risk factor, causing tissue damage and genetic changes linked to urothelial carcinoma (UC) and small cell neuroendocrine carcinoma. The pathogenesis of this rare disease remains unclear. Due to its rarity, the natural history of ureteral SCC is poorly understood. Prognosis is generally poor, and surgical interventions are not curative, but adjuvant chemotherapy may offer some potential for extending survival in affected patients. Herein, we present a case with primary SCC of the ureter and concurrent UC of the bladder from clinical presentation, image, management, pathology and follow-up condition after nearly a year.

This is a 55-year-old female patient with a history of hepatitis B, old pulmonary tuberculosis and right lower lung pulmonary nodules, caseating granulomatous inflammation s/p thoracoscopic wedge resection of lung about 3 years ago. She presented with right flank pain and hematuria for a week. Under the initial impression of right middle ureteral stone, the ureterorenoscopic lithotripsy was performed on 2023-01-11. During the operation, the right ureteral tumor with stone encrustation and bladder tumor over right lateral wall were found. The biopsy pathology reported SCC of right middle ureter and papillary urothelial carcinoma of bladder. The contrast MRI revealed urinary bladder neoplasm at right inferior wall(1.9cm), with focal muscular layer involvement, segmental right middle ureteral wall thickening and right hydronephrosis with perirenal infiltrations. Hand-assisted retroperitoneoscopic nephroureterectomy with bladder cuff excision and TURBT were performed on 2023-02-06. The patient was discharged uneventful five days after the operation. The pathology reported pure small cell carcinoma of the ureter with muscularis invasion(pT2), non-invasive urothelial papillary carcinoma of the bladder(pTa) and no lymph nodes metastasis. The patient received 12 cycles of the BCG instillation for bladder urothelial carcinoma and refused chemoradiotherapy for small cell carcinoma. No recurrence was noted in recent survey. Currently, the patient is still following-up in our outpatient department.