A Case of Amyloid of the Urethra and Review of this Rare Diagnosis, its Natural History and Management, with Reference to the Literature

Abstract

Primary localized genitourinary amyloid deposition is a rare disease that can be confused with cancer. Amyloid tumours of the urethra are exceptionally rare, with only 40 cases having been reported in the literature since 1909. A case is presented herein, with a full review of the presenting features, coexisting conditions and pathology and recommendations for treatment, based on the findings in previously reported cases.

Urethral Amyloidosis in a Patient with IgA Nephropathy After Renal Transplant

“…The lesion can be found with cystoscopy and in most cases of localized urethral amyloid tissue, the only treatment required is local excision of the tissue. Recurrence of a urethral amyloid is uncommon [4]. Once an amyloid tissue has been identified on histopathology, further workup to distinguish primary (also called systemic amyloidosis) versus secondary amyloidosis is recommended [3].…”

“…In a review of urethral amyloidosis case reports, known inflammatory conditions such as gonorrheal urethritis were identified in several cases, but a majority of cases had no associated inflammatory conditions [4]. To our knowledge, there is no known association between IgA nephropathy and amyloid deposition, although IgA nephropathy may have a systemic inflammatory component that could contribute to the development of amyloidosis.…”

“…It is found less commonly in the genitourinary tract, particularly in the urethra; fewer than 60 cases of urethral amyloidosis have been described in the literature [3-4]. Urethral amyloidosis can present at any age; but, it is mostly found in males with initial presenting symptoms including hematuria, dysuria, urethral discharge, penile induration or masses, and gross urethral bleeding [4-5]. While urethral amyloidosis is a rare condition, there is no known association between immunoglobulin A (IgA) nephropathy and amyloidosis.…”

Buccal mucosa graft urethroplasty in a case of urethral amyloidosis presenting with long anterior urethral stricture

“…They found recurrence of the lesion in only 2 cases, suggesting that the condition is selflimiting and that follow-up beyond 2 years is unnecessary. 2 Recently, the latest systematic review of the literature, in combination with personal experience, gave rise to a new perspective in the management of urethral amyloidosis. Mangera and colleagues described a progressive course of the disease in 3 of their 4 cases, when multiple repeated urethral procedures (urethrotomy, dilatation and intermittent self-dilation) can cause pan-urethral stricture disease.…”

“…The clinical presentation usually resembles obstructive voiding symptoms, while hematuria can raise a suspicion of malignancy. 2 Standard treatment has included a conservative approach and minimally invasive procedures without medium or long-term follow-up. Only a few cases of substitution urethroplasty have been reported, with good medium-term outcomes.…”