泌尿道局部類澱粉蛋白沉積症之臺中榮總經驗分享
廖博崎、賀昊中、裘坤元、陳卷書、洪啟峰、程千里、李建儀、王賢祥、楊晨洸、陳正哲、歐宴泉
臺中榮總外科部泌尿外科
Localized amyloidosis of the urinary tract - experience of VGHTC
Po-Chi Liao, Hao-Chung Ho, Kun-Yuan Chiu, Chuan-Shu Chen, Chi-Feng Hung, Chen-Li Cheng, Jian-Ri Li, Shian-Shiang Wang, Cheng-Kuang Yang, Cheng-Che Chen, Yen-Chuan Ou
Division of Urology, Department of Surgery, Taichung Veterans General Hospital
Purpose: Localized amyloidosis of the urinary tract is a rare condition. Due to its similar clinical presentation with malignancy of urinary tract, it is important for clinicians to recognize this condition.
Materials and Methods: We collect patients diagnosed as localized amyloidosis of the renal pelvis, ureters, urinary bladder, or urethra from 2000 to 2015. Patients with systemic amyloidosis were excluded and all cases were confirmed with Cango red staining. Initial presentation, image findings, treatment and outcome were analyzed.
Results: Five localized amyloidosis are identified. Among them, two of them are originated from urinary bladder and three from renal pelvis and ureter. None of our cases showed evidence of systemic amyloidosis. Painless gross hematuria is the most frequent symptom of localized amyloidosis of the urinary tract. Patients with bladder involvement were treated with localized bladder resection; those with renal pelvis and ureter involvement, two underwent ureteroscopic resection and one received percutaneous nephroscopic resection. After surgical resection, no complications were noted in patients with bladder involvement. Of patients with ureter and renal pelvis involvement, one require long-term double-J exchange for persisted obstructive uropathy, two developed permanent decrease of renal function.
Conclusions: Localized amyloidosis of the urinary tract is a rare condition that mimicking malignancy of the urinary tracts. To pathologic diagnosis of amyloidosis of the urinary tract, Congo red stain is essential. The treatment is usually conservative with observation or surgical resection with long-term follow up. Amyloidosis of renal pelvis and ureter cause more severe consequences than those with bladder involvement.