Primary localised amyloidosis of the urinary bladder: A recurrent and progressive disease

Abstract

Primary amyloidosis results from the deposition of amyloid protein fibrils in the extracellular space and rarely involves the urinary bladder. We present a 41-year-old man who was diagnosed with primary amyloidosis of the urinary bladder and underwent two sessions of transurethral resection of the bladder mass 4 years prior. Recently, the patient was admitted through the emergency with painless frank haematuria. Computed tomography of the abdomen and pelvis revealed a bladder mass that was larger than the previously reported mass. A repeat cystoscopy and resection of the mass was performed. Histopathological examination of the resected tissue revealed primary amyloidosis of the urinary bladder. A comprehensive examination was performed to exclude systemic amyloidosis.

Management of recurrent localised bladder amyloid, has methotrexate helped?

“…The aetiology of bladder amyloidosis is unknown. 3 This rare benign condition, classically presents in males in their sixth decade of life. 1 Haematuria, irritative voiding symptoms and abdominal pain traditionally thought to be the leading clinical presentations, were challenged in a recent publication by Sirohi et al in which the 29 patient case series had 62% of patients present with a bladder mass.…”

“… 5 Hence, initial evaluation for systemic amyloidosis is prudent, with echocardiogram, serum amyloid P scintigraphy, 24 hour protein electrophoresis, Bence Jones protein assessment and histology from abdominal subcutaneous fat, salivary gland or rectal mucosa. 3 …”

“…There is an emerging link between not only inflammatory arthropathy and amyloidosis but also treatment with disease modifying anti-rheumatic drugs such as methotrexate. 2 , 3 , 4 We hypothesise that this patient's amyloidosis was incidentally controlled with methotrexate and there may be a role for methotrexate in the bladder amyloidosis treatment algorithm.…”

“…Amyloidosis can be localised where amyloid deposits are produced and deposited in one isolated part of the body, or systemic where amyloidogenic protein produced at a distant site circulates in the blood depositing amyloid in distant tissues and resulting in organ dysfunction. 3 …”

“…Amyloidosis of the bladder is a benign condition, and accounts for around 1–2% of all bladder tumour aetiology. 3 It is primarily a disease of older males, presenting with haematuria or bladder mass, often requiring histological confirmation for diagnosis, with the use of a Congo red staining. 2 …”