Primary Localised Amyloidosis of the Urethra: A Case Report

Sachin Malde ,Nawal Khan ,Catherine Horsfield ,Kamran Ahmed ,Majed Shabbir

doi:10.31487/j.ajscr.2021.01.04

Abstract

Primary localised amyloidosis of the urethra is a rare entity with only 50 cases reported in the literature. The deposition of fibrillary proteins can lead to a range of symptoms. Importantly, the clinical and cystoscopic features may mimic urethral malignancy (haematuria, voiding difficulties, and a palpable urethral mass), and so thorough investigation is required in order to exclude malignancy, and to identify features of generalised amyloidosis which has a poor prognosis. Once diagnosed the prognosis of localised amyloidosis is excellent, although disease recurrence is possible. We describe a case of primary localised urethral amyloidosis presenting with visible haematuria and a urethral stricture and reviewed the literature regarding management of this rare condition.

Highlights

Amyloidosis is a rare group of diseases characterized by deposition of proteinaceous substances in the extra-cellular tissue
We describe a case of primary localised urethral amyloidosis presenting with visible haematuria and a urethral stricture and reviewed the literature regarding management of this rare condition
The generalised and localised patterns of the condition could be further sub-classified into primary amyloidosis which is associated with immunocyte dyscrasias (AL), or secondary which usually results from chronic inflammatory diseases or tissue destructive processes (AA) [1]

Summary

Introduction

Amyloidosis is a rare group of diseases characterized by deposition of proteinaceous substances in the extra-cellular tissue. The composition of these deposits may vary. We report a case of primary urethral amyloidosis associated with urethral stricture in a young male who presented with visible haematuria. Flexible urethroscopy demonstrated a white patch in the penile urethra with a proximal urethral stricture. He subsequently underwent urethral dilatation and biopsy of the lesion under general anaesthesia. After urethral dilatation a 19Fr cystoscope was used to examine the proximal urethra and the bladder He has had no deterioration in his flow at short-term follow-up with no systemic manifestations of amyloidosis

Discussion

Conclusion