自家腎移植術を施行した限局性尿管アミロイドーシスの1例 A CASE OF LOCALIZED AMYLOIDOSIS OF THE URETER TREATED BY RENAL AUTOTRANSPLANTATION

症例は44歳,男.左側腹部痛と肉眼的血尿を主訴とし受診.血尿を認める以外諸検査成績に異常はなかった.排泄性腎盂造影で,左尿管下部に長さ約10cmに及ぶ辺縁不整な狭窄像と,左水腎症を認めた.CTスキャンでは乏尿管壁の肥厚がみられたが,明らかな腫瘍像を認めなかった.左尿管狭窄と診断して手術施行.約15cmに及ぶ切除尿管の術中迅速病理学的検査で悪性像は認められず,自家腎移植術を施行した.術後,摘出尿管の病理学的診断はアミロイドーシスであった.術後検査により,血清蛋白分画正常,免疫グロブリン定量に異常なく,尿中Bence-Jones蛋白は陰性であることが判明した.腎および直腸生検ではアミロイド沈着を認めなかった.以上より限局性尿管アミロイドーシスと診断した.本症例は我々が集計し得た限りでは,世界で32例目で自家腎移植術施行例としては3例目であった.

A 44-year-old man presented with left flank pain and macrohematuria. The roentgenographic examination disclosed left hydronephrosis and stenosis of the left distal ureter. The patient was operated with the diagnosis of ureteral stricture. The frozen section during the operation revealed no malignancy. Therefore, autotransplantation of the left kidney was performed. Congo red staining revealed amyloid deposition in the resected segment of the ureter. No evidence of amyloidosis was obtained in rectal and renal biopsy. The other laboratory tests for amyloidosis were negative. Consequently, primary localized amyloidosis of the ureter was confirmed. Amyloidosis of the ureter is a rare entity. Review of the literature disclosed that this was the 32nd case in the world. Clinical features of localized amyloidosis of the ureter are similar to those of ureteral tumor. Since preoperative diagnosis is difficult, nephroureterectomy is usually performed for this disease. There were only two cases prior to ours which were treated by autotransplantation of the kidney. In the discussion, localized amyloidosis of the ureter was statistically analysed by reviewing the literature. The indication and advantages of autotransplantation of the kidney were also described.