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| | | Year : 2008 | Volume : 51 | Issue : 3 | Page : 415-417 | | | Localized amyloidosis of the urinary bladder, clinically masquerading as bladder cancer | | | Maria Alina DeSouza1, Bharat Rekhi1, Yuvaraja B Thyavihally2, Hemant B Tongaonkar2, Sangeeta B Desai1
1 Department of Pathology, Tata Memorial Hospital, Parel, Mumbai, India
2 Department of Surgical Oncology (Uro-Gynaecology), Tata Memorial Hospital, Parel, Mumbai, India
Click here for correspondence address and email | |      | |  Abstract | | | Amyloidosis is characterized by extracellular deposition of a proteinaceous, hyaline material. Localized deposition of amyloid in individual organs is uncommon. It can occur in the absence of systemic involvement. Herein, we describe two cases of localized amyloidosis of the urinary bladder, which clinically, radiologically, and on cystoscopy masqueraded as bladder cancer. A diagnosis of amyloidosis in both these cases was ascertained on biopsy, supplemented with special stains. Keywords: Amyloidosis, urinary bladder amyloidosis How to cite this article:
DeSouza MA, Rekhi B, Thyavihally YB, Tongaonkar HB, Desai SB. Localized amyloidosis of the urinary bladder, clinically masquerading as bladder cancer. Indian J Pathol Microbiol 2008;51:415-7 |
How to cite this URL:
DeSouza MA, Rekhi B, Thyavihally YB, Tongaonkar HB, Desai SB. Localized amyloidosis of the urinary bladder, clinically masquerading as bladder cancer. Indian J Pathol Microbiol [serial online] 2008 [cited 2014 Mar 6];51:415-7. Available from: http://www.ijpmonline.org/text.asp?2008/51/3/415/42547 |
| Introduction | |  |
Amyloidosis is a group of diseases characterized by the deposition of extracellular, hyaline, amorphous, proteinaceous material in various organs of the body. Progressive extracellular deposition of amyloid causes pressure atrophy of adjacent cells, ultimately resulting in loss of organ function. Deposition of amyloid can be localized (restricted to one organ or site) or systemic (involving many organs and tissues). [1],[2]
Localized amyloid deposition in the bladder is uncommon. [3],[4] We present two cases of localized bladder amyloidosis, which clinically, radiologically, as well as cystoscopically, mimicked bladder cancer.
| Case history | | |
Case 1
A 51-year-old lady referred to the urology outpatient department (OPD) with complaints of intermittent, painless, gross hematuria since 4 months. She denied history of any significant infectious disease; chronic illness, including significant joint pains. Clinical examination revealed no significant abnormality.
Laboratory investigations
Complete blood count (CBC) was within normal limits. Urine cytology revealed presence of RBCs and few degenerate "suspicious" cells.
Radiological findings
Ultrasound (USG) of the pelvis revealed two lobulated masses in the anterior bladder wall. CT scan of the abdomen and pelvis revealed a nodular growth measuring 5 cm × 1.9 cm and involving the anterior wall of the bladder. Perivesical fat was normal, and there was no evidence of extravesical spread.
Subsequently, the patient was posted for cystoscopy, which revealed presence of a large, sessile bladder mass measuring 5 cm × 6 cm; thereafter, transurethral resection of bladder tumor (TURBT) was performed.
Histopathological findings
Conventional hematoxylin and eosin (H and E)-stained sections revealed hyperplastic urothelium with lamina propria revealing presence of large deposits of eosinophilic, hyaline material, mostly deposited around blood vessels. Special staining (alkaline Congo red) revealed "salmon pink" appearance and, subsequently, "apple-green" birefringence under polarized light. A diagnosis of amyloidosis was offered.
A follow-up with cystoscopy and TURBT after 3 months revealed small recurrence of disease, which was resected transurethrally. The patient was disease free at 2 years of follow-up.
Case 2
An 82-year-old gentleman presented with complaints of painless hematuria since 1 year, which had become intermittent and gross since 1 month, accompanied with increased frequency of micturition. He was a known case of non-insulin-dependent diabetes mellitus (NIDDM), diagnosed 7 years back, and currently on antidiabetic treatment. Of late, he was also diagnosed as being hypertensive and had been on antihypertensive treatment. He also revealed history of being a chronic active tobacco chewer. He had undergone a cataract surgery in the recent past. There was no history of any significant infectious disease or joint pains. Clinically, no significant abnormality was noted. Per rectal examination revealed mild prostatic enlargement with no definite nodule. Genital examination revealed bilateral hydrocele.
Laboratory investigations
CBC and serum electrolytes were within normal limits. Blood sugar levels [fasting (FBSL) and postprandial (PPBSL)] were on the higher side of the normal range (FBSL, 107 mg/dL, PPBSL, 123 mg/dL). Electrocardiography (ECG) revealed "ST" changes in the inferior leads but normal left ventricular function. 2-D echocardiography revealed sclerotic aortic and mitral valve leaflets but a normal left ventricular ejection fraction of 55%. Urine cytology revealed presence of numerous RBCs with lack of malignant cells.
Radiological findings
Chest x-ray did not reveal any significant abnormality. A pelvic USG revealed an echogenic mass arising from the bladder. CT scan revealed a prominent prostate with obliteration of the neck of the urinary bladder causing right hydroureter and hydronephrosis.
Cystoscopy showed presence of a broad-based mass in the bladder neck and trigone, extending from the 3-o'clock to 9-o'clock position and measuring 3 cm × 3 cm. Urethra appeared normal. Transurethral resection of the tumor was performed.
Histopathological findings
Conventional H and E-stained microsections revealed amorphous pink material present diffusely and in the medial coat of vessels, along with plasma cells, eosinophilic, Russell's bodies, and foreign body-type giant cells. Alkaline Congo red staining revealed "salmon pink" appearance and further produced an "apple-green" birefringence under a polarizing microscope, confirming the diagnosis of amyloidosis [Figure 1].
Further, immunohistochemistry (IHC) was carried out with κ and 'λ light chain proteins, which did not reveal any restriction/clonality in the plasma cells.
The patient was lost for follow-up.
| Discussion | | |
Amyloidosis can be localized or systemic. The systemic form is subclassified into primary type, which is associated with an immunocyte dyscrasia; and secondary type, which occurs as a complication of an underlying chronic inflammatory process like rheumatoid arthritis, tuberculosis, etc. [1],[4] Localized amyloidosis, without systemic involvement, has been noted in the lung, larynx, skin, tongue and periocular area. [1] In the urinary tract, kidney is the more commonly involved organ in systemic or secondary amyloidosis (AA subtype), while urinary bladder is affected by primary localized disease. [5] Nonetheless, localized amyloidosis of bladder is uncommon, with less than 90 cases reported in the literature. [3],[4] The lesion assumes importance for discussion as it clinically masquerades as a malignancy, as seen in both of our cases.
Almost all documented cases, along with present cases, of bladder amyloidosis have been known to present with intermittent, gross hematuria, while a few patients presented with irritative bladder symptoms. [4],[6] Our second case presented with an increased frequency of micturition, which was probably secondary to non-insulin-dependent diabetes mellitus (NIDDM).
On cystoscopy, amyloidosis usually presents as an irregular, "tumor-forming," exophytic lesion that bleeds readily and may be ulcerated. Even on radiology, it presents as a mass lesion, sometimes associated with calcification. [5],[6] Both our cases presented radiologically and cystoscopically as mass lesions; the first case, in addition, revealed some papillary excrescences. Both these cases were thereby suspected to be primary urothelial carcinomas. The diagnosis of amyloidosis can only be established on biopsy, supplemented with special stains, as seen in our cases. [4],[5],[6]
On histopathology, presence of extracellular, eosinophilic, hyaline-like material, especially in the tunica media of vessels, led to suspicion of amyloid, as seen earlier. [5] Invariably, amyloid is seen dissecting collagen fibers of lamina propria, extending into the muscle layer. We did not identify muscle in the biopsy submitted. In view of presence of foreign body giant cells and chronic inflammation in the second case, possibility of fungal or parasitic lesion, including shistosomiasis, was included. However, lack of any organisms ruled out this possibility. Confirmation of pink material with alkaline Congo red stain led to an objective diagnosis of amyloidosis. Both the patients were thereafter investigated for any systemic manifestations like bony lesions, along with bone marrow examination, urinary Bence Jones proteins, and serum electrophoresis. Lack of systemic lesions ruled out multiple myeloma.
While the etiology of localized amyloidosis is not known, it is hypothesized that the deposits result from local synthesis of amyloid protein. [7] Frequently, there are infiltrates of lymphocytes and plasma cells in the periphery of these amyloid deposits, raising the question whether the mononuclear infiltrate is a response to the amyloid deposits or, instead, is responsible for it. At least in some cases, the amyloid consists of AL protein and may therefore represent a localized form of immunocyte-derived amyloid. [1] In our second case, amyloid deposition was accompanied with plasma cells, which, however, did not show light chain restriction, suggesting that the plasma cell infiltrate may be a response to, rather than a cause of, the amyloid deposit in our case.
Evidence of systemic involvement can be obtained by performing skin, rectal, kidney, and abdominal fat biopsies, as well as urinary Bence Jones proteins, serum electrophoresis, Venereal Disease Research Laboratory (VDRL) test, creatinine clearance, nerve conduction studies, ECG, USG abdomen; and x-ray spine, skull, and ribs. A newer technique called serum amyloid P component (SAP) scintigraphy can show specific uptake in organs such as liver, spleen, kidney, adrenals, etc. [2],[5],[6]
Therapeutically, surgery remains the mainstay of treatment, including fulguration or laser for small localized lesions and transurethral resection or partial cystectomy for larger lesions. [5],[6] Medical treatments such as intravesical dimethyl sulfoxide installation and oral colchicine have also been tried. [6] Both our cases underwent transurethral resection; and one, in addition, was treated by melphalan.
To sum up, localized amyloidosis should be considered as a differential diagnosis in suspected cases of bladder carcinomas, especially in cases with underlying chronic illnesses. Presence of amorphous material, with or without chronic inflammation, on the sections is a useful clue, which can be confirmed with special stains.
| References | | |
| 1. | Kumar VK, Abbas AK, Fausto N. Robbins and Cotran Pathologic Basis of Disease, In : Kumar VK, Abbas AK, Fausto N, editors. 7 th ed. Elsevier; 2004. p. 258-64.  | | 2. | Hazenberg BP, van Gameren II, Bijzet J, Jager PL, van Rijswijk MH. Diagnostic and therapeutic approach of systemic amyloidosis. Neth J Med 2004;62:104-5. | | 3. | Caldamone AA, Elbadawi A, Moshtagi A, Frank IN. Primary localized amyloidosis of urinary bladder. Urology 1980;15:174-81. | | 4. | Auge BK, Haluszka MM. Primary amyloidosis of the bladder. J Urol 2000;163:1867-8. | | 5. | Goswami AK, Vaidyanathan SS, Rao MS, Malik AK, Mathur RP, Sen TK. Primary localized amyloidosis of urinary bladder: A case report. J Postgrad Med 1984;30:253-4. | | 6. | Biewend ML, Menke DM, Calamia KT. The Spectrum of localized amyloidosis: A case series of 20 patients and review of literature. Amyloid 2006;13:135-42. | | 7. | Akram CM, Al-Marhoon MS, Mathew J, Grant CS, Rao TV. Primary localized AA type amyloidosis of urinary bladder: Case report of rare cause of episodic painless hematuria. Urology 2006;68:1343.e15-7. |
Correspondence Address:
Bharat Rekhi
Department of Pathology, Tata Memorial Hospital, Dr. Ernest Borges Road, Parel, Mumbai - 400 012
India
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DOI: 10.4103/0377-4929.42547 PMID: 18723977
[Figure 1] | |
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