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|Year : 1984 | Volume
| Issue : 4 | Page : 253-4
Primary localised amyloidosis of urinary bladder (a case report).
Goswami AK, Vaidyanathan SS, Rao MS, Malik AK, Mathur RP, Sen TK
|How to cite this article:|
Goswami A K, Vaidyanathan S S, Rao M S, Malik A K, Mathur R P, Sen T K. Primary localised amyloidosis of urinary bladder (a case report). J Postgrad Med 1984;30:253
|How to cite this URL:|
Goswami A K, Vaidyanathan S S, Rao M S, Malik A K, Mathur R P, Sen T K. Primary localised amyloidosis of urinary bladder (a case report). J Postgrad Med [serial online] 1984 [cited 2019 Nov 13];30:253. Available from: http://www.jpgmonline.com/text.asp?1984/30/4/253/5434
Amyloidosis is a disease complex resulting in acellular deposition of twisted beta pleated sheet fibrils formed from various proteins by different pathogenic mechanisms in different organs. The accumulation of these fibrils results in pressure atrophy and death of the affected organ. The tinctorial and optical properties of amyloid deposit on special staining are dependent on this beta pleated sheet configuration of the fibril.
The localised disease of an organ, like systemic disease, can be either primary or secondary. In the urinary tract, kidney is nearly always involved in "reactive amyloidosis" (secondary or AA amyloidosis) and in more than 50% of cases of immunocyte-derived amyloidosis primary or AL amyloidosis). In contrast, the urinary bladder is more usually affected by primary localised disease.
N.C., a 45 year old male, was admitted to the emergency urology service with intermittent, profuse, painless hematuria. There was no past history of any chronic, infective or inflammatory disease. Clinical examination revealed no abnormality. Urine analysis showed no other feature but numerous RBC's per high power field. His haemoglobin was 9 gm%. TLC, DLC and blood biochemistry for renal functions were within normal limits. Excretory urography showed normal upper tracts. Bladder was of good capacity with a small filling defect projecting into the lumen from the left side. Cystoscopy revealed a 1 x 2 cm sized, solid, nodular, circumscribed mass with a hemorrhagic surface arising from the bladder base just medial and above the left ureteric orifice. A provisional diagnosis of carcinoma bladder was made and a transurethral resection of the lesion was performed. On histopathology, there were massive deposits of structureless eosinophilic material in the sub-epithelial region. On congo red staining, the material exhibited. the classical apple-green birefringence under polarised light [Fig. 1] and [Fig. 2].
Subsequently, the case was investigated for the evidence of systemic amyloidosis. These included urine for Bence-Jones protein, serum protein, serum electrophoresis, liver function tests, VDRL, creatinine clearance, radiologic survey of spine, skull and ribs, sternal marrow biopsy and rectal biopsy. All were negative for amyloidosis, The patient is being followed-up for the last two years. He is asymptomatic. Check cystoscopies were normal and there is no evidence of systemic disease.
Primary localised amyloidosis is a rare disease. Only 38 cases have been reported in the English literature. Nearly all presented with intermittent, gross, painless hematuria. Irritative bladder symptoms were present in a few cases. Since clinical, radiological and cystoscopic features closely simulate bladder malignancy, it is not possible to establish a correct diagnosis of amyloidosis except by histopathological study of the biopsy from the vesical lesion. Clinical diagnosis has new been made.
Most cases of vesical amyloidosis appear to be of primary type-the so-called "tumour-forming" amyloidosis. The etiology of this type of amyloidosis is unknown. The bladder wall is thickened and mucosa is hyperemic, rough, nodular with small ulcerations which simulate sessile carcinoma on cystoscopy. Histologically, amyloid deposit is typically amorphous and more or less homogenous. It is mainly seen in and between the collagen fibres of lamina propria but can extend into the muscularis. There is predilection for involvement of the media of arteries and veins. Muscle cells are replaced by amyloid material. Polarization of amyloid stained with Congo red gives apple-green birefringence. It appears bluish-green on staining with sulphonated aniline blue (SAB) stain.
Before vesical amyloidosis is classified as primary, a diligent search should be made to unmask any possible etiological disorder (e.g., chronic suppuration, tuberculosis) and amyloid deposits elsewhere in the body. Rectal, liver and skin biopsies, subcutaneous fat aspirate and serum electrophoresis will usually provide evidence regarding systemic involvement. Congo red staining and electron microscopy of body fluid's like synovial fluid and urine may not provide unequivocal evidence, as patients with amyloidosis and non-amyloidotic proteinuria as well as normal persons have had urinary sediments that stained with congo red in the classic manner of fibrillar Components.
Massive haemorrhage clue to vesical amyloidosis may be controlled by intravesical instillation of formalin. Definite treatment modalities include transurethral resection, partial or total cystectomy with urinary diversion depending on the extent of vesical involvement. Small lesions can be successfully treated by transurethral resection. Although recurrences and/or progression have been reported after transurethral resection, our patient has been unaffected thus far during a 2 year follow-up phase.
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