病例報告: 輸尿管類澱粉沉積症引發之嚴重腎積水

李虹霈¹、李香瑩¹

高雄醫學大學附設醫院 泌尿部 ¹

Case report: A rare case of ureteric amyloidosis causing severe hydronephrosis

Hung-Pei Li¹、 Hsiang-Ying Lee ¹

¹Department of Urology, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan.

Abstract

  Here we present this case with a focus on sharing the clinical and image manifestation of ureteric amyloidosis, which was a rare entity and difficult to be differentiated from malignancy based on preoperative image survey.

A 62-year-old female with a medical history of hypertension and ischemic stroke, first presented with right flank pain for weeks. Sonography showed right hydronephrosis, grade 4. Intravenous urography revealed severe delay function and opacification of the right collecting system. Abdominal computed tomography disclosed severe right hydronephroureter and calcification at right lower renal calyx. Urine cytology was negative for high-grade urothelial carcinoma. Right ureteroscopy survey showed severe tortuous and stricture of the right ureter with an uneven surface. Biopsy of the uneven surface was performed.  Under microscopic examination the urethral specimen showed submucosal amorphous pale eosinophilic deposition with kappa light chain restriction demonstrated by kappa and lambda chain stains.  Apple-green birefringence with polarized light is identified by congo red stain, consistent with the diagnosis of ureteric amyloidosis.  Due to difficulty in ureteral stent placement under ureteroscopy, right Percutaneous Nephrostomy (PCN) insertion was arranged for severe hydronephrosis.