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CASE REPORT Table of Contents   
Year : 2008  |  Volume : 51  |  Issue : 2  |  Page : 247-249
Localized amyloidosis of urinary bladder: A diagnostic dilemma


Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India

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   Abstract 

Amyloidosis is a heterogeneous group of disorders affecting a single-or multiple-organ system and presents as generalized or localized disease. Both generalized amyloidosis and localized amyloidosis can be primary or secondary. Localized amyloidosis affects organs like urinary bladder, lung, larynx, skin, tongue and the region around the eye, producing detectable nodular masses which are clinically suspected as malignancy. We present six cases of localized urinary bladder amyloidosis that were clinically and cystoscopically suspected as bladder tumor or cystitis, which occurred over a period of last 10 years. Histology in all cases revealed diagnosis of primary amyloidosis. None of them had any stigmata of secondary disease. The cases were treated by simple transurethral resection of bladder. Two out of the six cases recurred after 3 to 5 years of initial presentation and were asymptomatic thereafter. Amyloidosis of the bladder is a rare condition which often mimics bladder neoplasm clinically and cystoscopically and histological examination is a must for definite diagnosis and proper management.

Keywords: Amyloidosis, localized, urinary bladder

How to cite this article:
Jain M, Kumari N, Chhabra P, Gupta RK. Localized amyloidosis of urinary bladder: A diagnostic dilemma. Indian J Pathol Microbiol 2008;51:247-9

How to cite this URL:
Jain M, Kumari N, Chhabra P, Gupta RK. Localized amyloidosis of urinary bladder: A diagnostic dilemma. Indian J Pathol Microbiol [serial online] 2008 [cited 2014 Dec 18];51:247-9. Available from: http://www.ijpmonline.org/text.asp?2008/51/2/247/41671



   Introduction Top


Amyloidosis encompasses a heterogeneous group of disorders which are characterized by extracellular deposition of eosinophilic fibrillar protein in various tissues and organs. It can be classified based on clinical syndromes (generalized/systemic or localized) or based on constituent protein fibrils (amyloid light chains - AL; amyloid associated protein - AA; or transthyretin - ATTR). Clinically generalized amyloidosis can be subclassified into primary when associated with immunocyte dyscrasia or secondary when it occurs as a complication of chronic inflammatory disorder. Localized amyloidosis, like systemic, can also be primary or secondary. The organs commonly involved are urinary bladder, lung, larynx, skin, tongue and the region around the eye; and the deposits produce detectable nodular masses, which may result in pressure atrophy and death of the affected organ. Hereditary or familial group constitutes a separate heterogeneous entity, with several distinct patterns of involvement.

In the urinary tract, deposition of amyloid can occur anywhere, starting from kidney to renal pelvis, ureters, urethra and even penis. The kidney is nearly always involved in secondary amyloidosis and in approximately 50% of the cases of primary amyloidosis. This is in contrast to the urinary bladder, which is usually affected in primary localized amyloidosis. [1],[2],[3] Primary localized amyloidosis of urinary bladder is quite rare; and according to Malek et al. , in 2002, there were only 160 cases reported in the literature. [4] However, quite a few publications have appeared in literature thereafter, including the one earlier reported from our institute. [5]

Amyloidosis is a rare cause of irritative voiding symptoms and hematuria and presents as an uncommon differential diagnosis of urinary bladder carcinoma. Primary amyloidosis localized to the urinary bladder is quite rare. [4],[6],[7] Patients usually present with gross hematuria, irritative voiding symptoms, or both. Localized amyloidosis poses a diagnostic problem because of its nonspecific presenting symptoms and uncharacteristic cystoscopic appearance, as well as radiological appearances which may mimic inflammatory lesion or a neoplastic lesion. Histopathological examination is necessary to confirm the diagnosis of amyloidosis and to exclude malignancy. The amyloid deposits are congophilic and have an apple green birefringence when viewed on polarized microscopy. The composition of deposits can be demonstrated immunohistochemically. AL and AA amyloid deposits can be distinguished to some extent histochemically by treating with potassium permanganate (KMnO 4 ). The primary deposits (AL) usually persist after KMnO 4 treatment, whereas secondary (AA) deposits are digested. Deposits of primary amyloidosis typically involve submucosa and adjacent superficial smooth muscle of the urinary bladder. In contrast, deposits in secondary amyloidosis involve more commonly the blood vessels. [4] This explains why secondary amyloidosis presents more commonly with gross hematuria.

We report a series of six cases in which there was diagnostic dilemma clinically. Five of them were suspected to have carcinoma bladder, whereas one was suspected to be a case of chronic cystitis. Surprisingly, all of them turned out to be localized amyloidosis of urinary bladder on histopathology.


   Case History Top


The details of cases, their clinical data and follow-up were retrieved from the records of the Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow. Cystoscopic examination, urinary cytology and transurethral retrograde bladder biopsy were done in every case. The tissues were embedded for paraffin blocking and routine hematoxylin-eosin stain was performed on 5-µ thick sections. Congo red staining was performed on 8- to 9-µ thick sections with and without KMnO 4 . Urine culture and sensitivity, serum biochemistry, serum electrophoresis, rectal biopsy, abdominal fat pad biopsy and bone marrow examination were performed in all cases.


   Results Top


Six cases of amyloidosis of urinary bladder were retrieved over a period of 10 years. The patients were four men and two women and their age ranged between 35 and 62 years. Hematuria was the predominant symptom in five cases, whereas the main complaint in one case was dysuria. Case Nos. 1 and 4 showed multiple nodules studded in the entire bladder. Case Nos. 2, 3 and 5 showed single mass, whereas case No. 6 showed diffuse irregular thickening of the bladder wall. Urine culture and sensitivity were sterile in all cases. Serum electrophoresis, liver enzymes, serum electrolytes and findings from bone marrow examination were within normal range. Rectal biopsy and abdominal fat pad biopsy for amyloidosis and urine cytology for malignant cells were negative [Table 1].

Histopathological examination of bladder biopsies in all cases revealed extensive deposits of eosinophilic acellular material beneath the surface epithelium, within the lamina propria, in the muscularis propria and intramurally within the vessel wall. The deposits were brightly congophilic and gave an apple green birefringence on polarized light microscopy. Congophilia persisted even after treatment with KMnO 4 [Figure 1],[Figure 2]. Electron microscopy done in two cases showed nonbranching thin fibrils. Thus all patients had a diagnosis of primary urinary bladder amyloidosis. In all cases, rectal biopsy and amyloid fat pad aspiration cytology were done to rule out systemic amyloidosis, which were negative. All cases were treated by simple transurethral resection of bladder. Follow-up was available for 1 to 9 years. All the patients were found to be asymptomatic except for the case Nos. 1 and 3, which recurred after 3 and 5 years respectively [Table 2].


   Discussion Top


Amyloidosis localized to the urinary bladder is a rare condition; and from India, a few cases have been reported. [1],[4],[5] In the present study, all the cases had localized amyloidosis of urinary bladder and recurrence occurred in two cases. All the cases clinically had a provisional diagnosis of carcinoma of urinary bladder. Gross hematuria with or without dysuria is the most common clinical presentation of malignancy, as well as of localized amyloidosis. [8] Five cases in the present study presented with hematuria; and of which two cases had, in addition, dysuria. The remaining one case presented with dysuria as the chief complaint. Primary systemic amyloidosis is usually not accompanied by lower urinary tract symptoms, whereas primary localized bladder (tumor-forming) amyloidosis is almost always accompanied by such clinical features. [4],[9] The incidence of gross hematuria has been reported to be 58% to 77% and irritative voiding symptoms have been reported in 23% of cases in different studies. [4],[10]

All our cases presented as nodular-to-polypoidal, single or multiple masses on cystoscopic examination. Histopathology of these lesions showed deposits of amyloid. This is a very rare condition of gross hematuria and not commonly thought of clinically as a differential diagnosis of malignancy. None of our cases developed associated malignancy, though reports of malignancy coexisting with amyloidosis of bladder are present. [10] On follow-up for about 1 to 9 years, all the patients were found to be asymptomatic except for the case Nos. 1 and 2, in which there was recurrence after 3 and 5 years respectively with a similar histological picture as before but they were asymptomatic thereafter.


   Conclusion Top


Localized amyloid of bladder is a rare disease. The age of onset is the same as that of urinary bladder tumors (sixth to eighth decade) and cystoscopic findings are also similar in the form of nodular or polypoidal masses; therefore, histological examination is mandatory. A clinically suspicious negative cystoscopic bladder biopsy should be cautiously evaluated for the presence of amyloidosis and histologically suspected cases of amyloid should be subjected to congo red staining. Any recurrences after first diagnosis of amyloid should also be assessed thoroughly, as malignancies are known to occur simultaneously or develop subsequently after amyloidosis.

 
   References Top

1.Goswami AK, Vaidyanathan SS, Rao MS. Primary localized amyloidosis of urinary bladder: A case report. J Postgrad Med 1984;30:253-4.  Back to cited text no. 1    
2.Dias R, Fernandez M, Patel RC, de Shadarevian JJ, Lavengood RW. Amyloidosis of renal pelvis and urinary bladder. Urology 1979;14:401-4.  Back to cited text no. 2    
3.Strong GI, Kelsey D, Hoch W. Primary amyloid disease of bladder. J Urol 1974;112:463-6.  Back to cited text no. 3    
4.Malek RS, Wahner-Roedler DL, Gertz MA, Kyle RA. Primary localized amyloidosis of the bladder: Experience with dimethyl sulfoxide therapy. J Urol 2002;168:1018-20.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Zaman W, Singh V, Kumar B, Mandhani A, Srivastava A, Kumar A, et al. Localized primary amyloidosis of the genitourinary tract: Does conservation help? Urol Int 2004;73:280-2.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]
6.Ferch R, Haskell R, Farebrother T. Primary amyloidosis of urinary bladder and ureter. Br J Urol 1997;80:953-4.  Back to cited text no. 6  [PUBMED]  
7.Grainger R, O'Riordan B, Cullen A, Kelly D, Heaney J. Primary amyloidosis of lower urinary tract. Urology 1988;31:14-6.  Back to cited text no. 7  [PUBMED]  
8.Lehtonen T, Makinen J, Wikstrom S. Localized amyloidosis of urinary bladder. Eur Urol 1991;20:113-6.  Back to cited text no. 8    
9.Tirzaman O, Wahner-Roedler DL, Malek RS, Sebo TJ, Li CY, Kyle RA. Primary localized amyloidosis of the urinary bladder: A case series of 31 patients. Mayo Clin Proc 2000;75:1264-8.  Back to cited text no. 9  [PUBMED]  
10.Khan SM, Birch PJ, Bass PS, Williams JH, Theaker JM. Localized amyloidosis of the lower genitourinary tract: A clinicopathological and immunohistochemical study of nine cases. Histopathology 1992;21:143-7.  Back to cited text no. 10  [PUBMED]  

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Correspondence Address:
Manoj Jain
Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow - 226 014
India
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DOI: 10.4103/0377-4929.41671

PMID: 18603696

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    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1], [Table 2]

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    Abstract
    Introduction
    Case History
    Results
    Discussion
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