|Year : 2003 | Volume
| Issue : 2 | Page : 157-159
Primary amyloidosis of the urinary bladder: A case report
N Srinath1, AS Sandhu2, R Bharadwaj2, R Sood2, SK Gupta2, AA Pradhan2, P Madhusoodanan2
1 Department of Urology, Military Hospital, Jalandhar Cantt, India
2 Department of Urology and Pathology, Army Hospital (Research & Referral), Delhi Cantt, India
Department of Urology, Military Hospital, Jalandhar Cantt - 144 005
Source of Support: None, Conflict of Interest: None
| Abstract|| |
A case of young woman with primary bladder amyloidosis presenting with haematuria is reported. The diagnosis was confirmed by bladder biopsy. Secondary amyloidosis was excluded after extensive search for underlying causes. This rare entity can usually be treated conservatively. Long-term follow-up is recommended.
Keywords: Amyloidosis, urinary bladder, neoplasm, benign. Congored, haematuria.
|How to cite this article:|
Srinath N, Sandhu A S, Bharadwaj R, Sood R, Gupta S K, Pradhan A A, Madhusoodanan P. Primary amyloidosis of the urinary bladder: A case report. Indian J Urol 2003;19:157-9
|How to cite this URL:|
Srinath N, Sandhu A S, Bharadwaj R, Sood R, Gupta S K, Pradhan A A, Madhusoodanan P. Primary amyloidosis of the urinary bladder: A case report. Indian J Urol [serial online] 2003 [cited 2020 Oct 1];19:157-9. Available from: http://www.indianjurol.com/text.asp?2003/19/2/157/37149
| Introduction|| |
Primary amyloidosis of the urinary bladder is a rare entity with less than 90 cases reported.  The condition is of interest because it resembles a neoplasm both in symptoms and gross appearance. Accurate diagnosis depends on biopsy of the bladder lesion. The treatment is usually conservative, i.e., observation or transurethral resection. We report a further case of primary bladder amyloidosis.
| Case Report|| |
A 30-year-old woman presented in September 1999 with the complaints of occasional painless gross haematuria since the past 6 years and infertility. She had undergone transurethral resection of bladder tumour (TURBT) elsewhere in 1995. Histopathological examination was reported as bladder amyloidosis. She was empirically given anti-tubercular therapy. Check cystoscopy and TURBT for residual lesions in right lateral and posterior bladder walls was done 8 weeks later. Further details of treatment were not available. General, systemic and gynecological examination done in this centre was normal. Routine haematological and biochemical investigations were normal. Urine cytology and urine culture were normal. Urine examination for acid-fast bacilli in multiple samples was negative. Urine cultured for Mycobacterium tuberculosis s negative as was blood ELISA. Intravenous urogram and chest radiograph were normal. Pelvic ultrasound demonstrated multiple bladder tumours in posterior and lateral walls. Other pelvic organs were normal. 24-hour urinary protein excretion was 99 mg. Rheumatoid factor and Bence Jones protein were negative. Serum electrophoresis of liver function tests were normal. Echocardiography, abdominal fat pad aspiration, rectal and gingival biopsies were normal.
The patient underwent cystoscopy and TURBT. There were multiple yellowish, sessile tumours over posterior and both lateral bladder walls. Histological examination showed extracellular and perivascular deposits of amorphous eosinophilic material in the submucosa and muscle. There was a mild lymphocytic response. The transitional epithelium was unremarkable. The deposits after staining with Congo red showed apple green birefringence under polarizing light thus confirming the diagnosis of amyloidosis [Figure - 1] A and B. Diagostic workup for infertility revealed pelvic endometriosis and tubal adhesions. The patient was treated accordingly. The patient has been followed up for a year. There has been no recurrence of the bladder lesions or haematuria.
| Comments|| |
The etiology of amyloidosis is unknown., Immunological mechanisms are considered to play a role. In the case of bladder amyloidosis the postulated pathogenic mechanism is that chronic and recurrent mucosal and submucosal inflammation leading to chronic cystitis causes an influx of lymphoplasmacellular elements one of which becomes monoclonal. The monoclone proliferates and secretes an aberrant type of light polypeptide chain, which is amyloidogenic. By means of lysosomal proteolysis of phagocytic cells or other physiochemical means, these light chains are formed into amyloid fibrils and deposited in the bladder tissue. 
The condition presents a diagnostic challenge to the urologist since it commonly mimics transitional cell carcinoma due to similar symptoms of irritative voiding, pelvic pain and haematuria. Symmer's classification divides amyloidosis into:
(a) Secondary amyloidosis, which is associated with a chronic inflammatory condition such as rheumatoid arthritis, chronic osteomyelitis or a neoplasm such as multiple myeloma, and
b) Primary amyloidosis, which is not associated with any predisposing disease. It too may occur either in the generalised or localised tumefactive form. The latter has been reported in diverse sites, particularly, the respiratory tract, skin, mucus membranes and heart. In the genitourinary tract involvement of renal pelvis, ureter, bladder, prostate, seminal vesicles, urethra, penis and testes may occur.  Primary bladder amyloidosis with associated ureteric obstruction has also been described. , Our patient fits into the category of localized primary bladder amyloidosis.
Both sexes are affected equally in primary bladder amyloidosis with age ranging from 31 to 80 years (mean 51 years). There are no pathognomonic radiological features and a filling defect similar to a bladder tumor may be found.  Cytoscopic findings may resemble a malignant tumor with raised erythematous, papillary or haemorrhagic lesions.  Amyloid deposits may appear as yellowish submucosal plaques as in our patient. The diagnosis may be missed on routine histological examination unless special staining with Congo red is done.
Once the biopsy confirms amyloidosis a thorough search for possible underlying disease and associated secondary amyloidosis should be made. Primary bladder amyloidosis is generally treated conservatively with transurethral resection and fulguration. Medical treatment with substances such as colchicines,  nitrofuazone and dimethyl sulfoxide  as primary or adjuvant therapy have been tried. Cystectomy may be required in diffuse bladder involvement.  Procedures such as ligation of internal iliac arteries or cystectomy are occasionally necessary for control of massive haemorrhage in secondary bladder amyloidosis. The difference in behaviour can be explained by the predominance of blood vessel involvement in secondary as compared with predominantly submucosal and muscular involvement in primary disease. This results in the inability of these vessels to contract and thereby reduce haemorrhage. It also decreases tissue oxygenation by diffusion leading to increased tissue hypoxia and a greater tendency for mucosal ulceration in secondary cases.  Symptomatic local recurrence is common and usually amenable to repeat transurethral resection.  Long-term follow-up is recommended. 
The present case is unique in that to our knowledge primary bladder amyloidosis has not been described in a patient as young as ours (25 years at first presentation). The association with endometriosis appears incidental.
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[Figure - 1]