|Year : 2002 | Volume
| Issue : 2 | Page : 168-169
Primary localized amyloidosis of urinary bladder : A case report
Anil Kumar Goswami, Purshotam Kumar Puri, Bishan Dass Radotra, Shrawan Kumar Singh, Surinder Kumar Sharma
Department of Urology and Pathology, Postgraduate Institute of Medical Education & Research, Chandigarh, India
Anil Kumar Goswami
Department of Urology, Postgraduate Institute of Medical Education & Research, Chandigarh - 160 012
Source of Support: None, Conflict of Interest: None
Keywords: Primary Amyloidosis; Localized Amyloido-sis; Urinary Bladder Amyloidosis; AL-Type Amyloidosis.
|How to cite this article:|
Goswami AK, Puri PK, Radotra BD, Singh SK, Sharma SK. Primary localized amyloidosis of urinary bladder : A case report. Indian J Urol 2002;18:168-9
Primary localized amyloidosis of the urinary bladder is a rare disease, of unknown etiology and no standard treatment has been established so far. ,, We report a case of primary localized amyloidosis of the urinary bladder diagnosed by absence of amyloid deposition elsewhere and immunoperoxidase stain showing immunoreactivity to anti-AL antibodies.
| Case Report|| |
G.R. a 42-year-old male, known diabetic type-II for the last 7 years on oral hypoglycemic drug, presented with history of two episodes of painless, gross, total haematuria. Clinical examination was unremarkable. Routine investigations like haemoglobin, total and differential leukocyte counts, erythrocyte sedimentation rates, blood sugar, blood urea, serum creatinine, electrolytes, liver associated enzymes, electrocardiogram and chest X-rays were normal. Urine culture was negative. Voided urinary cytology for malignant cells was negative. An excretory urogram demonstrated normal urinary tract. Ultrasound KUB revealed minimal bladder wall irregularity near the bladder neck.
At cytoscopy, an irregular, haemorrhagic, oedematous lesion involving the trigone was noted. Lesion was resected. During resection submucosal yellowish deposits were seen [Figure - 1].
The biopsy specimen revealed deposits of pale, acellular, amorphous material, involving subepithelial area of bladder, having apple-green birefringence under polarized light after staining with congo-red. There was no evidence of malignancy. Using the potassium permanganate pretreatment method and immunohistochemical staining, the amyloid protein was characterized as a immunoglobulin of AL-type (composite [Figure - 2]). Investigations for systemic amyloidosis - Bence Jones protein assay, serum electrophoresis, rectal biopsy, abdominal fat pad aspiration, buccal mucosal biopsy, chest radiograph, echocardiogram, liver were all negative. On follow-up upto 9 months, there was no recurrence.
| Discussion|| |
Primary amyloidosis of the bladder is a rare clinico-pathological entity of unknown etiology. The condition is of interest to the urologists since the signs and symptoms resemble those of neoplasm. The major presenting symptom is gross haematuria, though some patients experience lower urinary tract symptoms such as increased urinary frequency, dysuria and lower abdominal pain or discomfort. 
A definite diagnosis of the disease can be made only by histological examination of biopsy material in which apple-green birefringence can be recognized under polarized light after staining with congo-red. There have been few reports on the immuno-histochemical type determination of amyloid protein in primary localized vesical amyloidosis. The pathogenesis of amyloid deposition is still not clear. A thorough search for possible underlying disease and associated secondary amyloidosis should be made.
Most of the reported patients with primary localized amyloidosis of the bladder have been treated with transurethral resection. Cystectomy has been performed in few cases with uncontrollable haematuria and diffuse lesions. Dimethyl sulfoxide has been successfully used both orally as well as by bladder instillation.  Because of the risk of recurrence, a close follow-up is mandatory.
| References|| |
|1.||Auge BK, Haluszka MM. Primary amyloidosis of the bladder. J Urol 2000; 163: 1867-1868. |
|2.||Kono M. Komatsu K. Nagasaka Y, Namiki M. Primary A-type amyloidosis of the urinary bladder: A case report. Hinyokika Kiyo 2000; 46: 197-199. |
|3.||Tokunaka S, Osanai H, Morikawa M, Yachiku S. Experience with Dimethyl Sulfoxide treatment for primary localized amyloidosis of the bladder. J Urol 1986: 135: 580-582. |
[Figure - 1], [Figure - 2]