Management and evaluation of localized amyloidosis of the bladder: Two case reports

Management and evaluation of localized amyloidosis of the bladder: Two case reports

CASE REPORT MANAGEMENT AND EVALUATION OF LOCALIZED AMYLOIDOSIS OF THE BLADDER: TWO CASE REPORTS LAURA E. CROCITTO, M.D. JAMES A. EASTHAM, M.D. LINDA ...

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CASE REPORT

MANAGEMENT AND EVALUATION OF LOCALIZED AMYLOIDOSIS OF THE BLADDER: TWO CASE REPORTS LAURA E. CROCITTO, M.D. JAMES A. EASTHAM, M.D. LINDA ZIEN, M.D• ElLA C. SKINNER, M.D. JEFFRY L. HUFFMAN, M.D. From the Department of Urology, University of Southern California, Los Angeles, California

ABSTRACT--Localized amyloidosis of the bladder is a rare cause of hematuria, which is often confused with bladder cancer. We present case histories of two patients which illustrate the evaluation and management of this entity. The diagnosis is usually made with biopsy and subsequent pathologic examination. Conservative management is attempted initially but must be individualized according to the clinical course of each patient. Transurethral resection will suffice in most instances but occasionally cystectomy is indicated to control local disease.

Amyloidosis is characterized by the deposition of a homogeneous, eosinophilic, hyaline protein in one or more body sites.l,2 Primary amyloidosis of the bladder is a rare condition that often mimics bladder cancer on initial presentation. 1,3-5It is important to differentiate primary- amyloidosis of the bladder from systemic amyloidosis and bladder cancer, since the management and outcomes are different. Systemic amyloidosis may be familial, idiopathic, or associated with various underlying inflammatory disease processes. Systemic amyloidosis rarely may present with gross hematuria. This is due to diffuse infiltration of the bladder by amyloid and should respond to bladder irrigation, but occasionally may require ligation of the hypogastric vessels for massive hemorrhage. Treatment of secondary systemic amyloidosis also includes treatment of the underlying disease process. Idiopathic systemic amyloidosis may be treated with various agents, including melphalan and prednisone, colchicine, and dimethyl snlfoxide. The long-term survival remains poor, with death secondary to cardiomyopathy and uremia. 6-1° Primary amyloidosis, on the other hand, when treated conservatively with transurethral resection, Submitted: January 20, 1994, accepted (with revisions): April 1, 1994

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has an excellent prognosis. Local recurrence is common but usually amenable to subsequent transurethral resection. Occasionally, cystectomy may be required to control local disease. >5 We present two patients with primary amyloidosis of the bladder. The first patient responded successfully. to transurethral resection alone. The second pa~ tient was m a n a g e d i n i t i a l l y w i t h multiple: transurethral resections, but subsequently required a cystectomy for recurrent hematuria. CASE REPORTS CASE 1

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A 61-year-old Hispanic man was admitted witk~ complaints of gross hematuria, frequency, and::~ dysuria. The patient had one similar episode 0f;:: gross hematuria 2 years prior to admission that re;: solved spontaneously. He gave a history of chrome-,_ prostatitis but no other past medical or surgi~a~i history. He denied tobacco use. The physical examination was normal. Laboratory values wer~ significant for a hematocrit of 39% (42% to 52%k blood urea nitrogen of t0 mg/dL (5 to 25 mg/dL)!~ and a creatinine of 1.2 mg/dL (0.5 to 1.3 mg/dL)!i An intravenous urogram revealed normal kidneyS: and u p p e r tracts. There w e r e multiple f!l~. ling defects within the bladder and an irregularit~ on the right lateral bladder wall. At cystoscoPY UROLOGY / AUGUST 1994 / VOLUME44, Nu'~B~2

The walls of these smafl blood vessels are thickened and expanded by amorphous, slightly fibrillar material that is suggestive of amyloid on routine hematoxylin and eosin stained sections (×40). FIGURE 2.

Cystoscopic view showing multiple yellow ubmucosat lesions in the bladder characteristic of (lens, 5 °).

;IGURE 1.

multiple clots were evacuated. A 2 x 3 cm area of vhat appeared to be infiltrating bladder cancer was dentified on the right posterior lateral bladder wall md transurethral resection was performed. Pathoogle review of the resected specimen showed only he presence of amyloidosis. An extensive workup br secondary causes of amyloidosis was negative. [his i n c l u d e d s e r u m a n d u r i n e p r o t e i n elec:roph0resis, serum immunoglobulin levels, serum :omplement, a Venereal Disease Research Labora:dry test (VDRL), rheumatoid factor, a tuberculin ikin:test, and rectal biopsy. C o m p u t e d tomogra,}iy and magnetic resonance scans were unre}~arkable. In v i e w of the n e g a t i v e s y s t e m i c }orkup, primary amyloidosis of the bladder was ~lagnosed. Postoperanvely, the patient has had no ~ccurrence of gross hematuria He has remained [¢ystosCopically free of disease for 29 months. !i least" 2 i! A 55-year-old Philippino m a n was referred for }valuation of recurrent bladder cancer. The patient resented initially in t981 with gross hematuria nd was diagnosed with superficial transitional cell ~arcinoma of the bladder He underwent multiple ~ansurethral resections over the next several years ~r,, recurrent superficial disease. In 1987 the pa~ent presented with gross painless hematnria, and ~ystoscopy revealed multiple yellow submucosal i~sionS (Fig. 1). Biopsies were done and the bases ~

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On staining with Congo red, amorphous salmon-pink areas are highlighted that show the characteristic apple-green birefrigence on polarization (×40).

FIGURE 3.

months later with gross hematuria and massive recurrence of the amyloidosis, which was resected. He p r e s e n t e d again 4 m o n t h s later with gross hematuria and recurrent disease and again underwent a transurethral resection. Due to the persistent hematuria and poor control of local disease, the patient underwent a cystectomy and continent Kock pouch diversion to the prostatic urethra. The patient has done well since and remains free of disease 42 months following surgery. COMMENT Primary amyloidosis of the bladder is a rare condition that often mimics bladder cancer in its initial presentation. Approximately 80% of patients present with gross, painless hematuria. 4 Frequency,

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dysuria, and suprapubic pain were noted in 40% of cases. 4 The lesion of primary amyloidosis in the bladder may be mistaken for bladder cancer by cystoscopic examination. It is usually broad based or polypoid with surrounding edema and ulceration.l~ The definitive diagnosis is based on the histopathologic examination of the resected specimen or biopsy. Amyloid is an eosinophilic, extracellular hyaline substance that is ubiquitous in distribution. It stains pink with the hematoxylin-eosin stain (Fig. 2). The Congo red stain is used to differentiate amyloid from other hyaline deposits, such as collagen or fibrin. When stained with Congo red and viewed under a polarizing microscope, amyloid, but not other hyaline deposits, will demonstrate a characteristic apple-green birefringence (Fig. 3). n'12 Once the diagnosis of amyloidosis is made, an extensive search for secondary systemic causes should be performed. Several chronic diseases are associated with amyloidosis, including chronic granulomatous disease, rheumatoid arthritis, multiple myeloma, osteomyelitis, inflammatory bowel disease, and metabolic diseases, such as diabetes mellitus} 1 Routine screening tests should include a complete blood count, chemistry panel, a urine culture, serum and urine protein electrophoresis, rheumatoid factor, tuberculin skin test, and VDRL. In addition, biopsy of the rectum, skin, esophagus, or larynx should be performed because 80% of patients with secondary amyloidosis will be positive for amyloid on the biopsy specimen, n If all of these studies are negative, the diagnosis of primary amyloidosis of the bladder can be made. If any of the tests are positive, a systemic disorder should be suspected and treated accordingly. The treatment of primary amyloidosis of the bladder should be conservative, since there is no evidence to indicate potential for malignant degeneration. Most cases are easily managed with transurethrat or segmental resection, which is curative in 80% of the patients. Close follow-up with frequent cystoscopy is required to detect recur-

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rence or any subclinical disease known to predispose to amyloidosis. Recurrent disease is ofte~i m a n a g e d effectively w i t h a subsequen[i transurethral resection. If recurrence cannot be~ controlled or if obstruction of a ureteral orifice o¢;i curs, then more aggressive procedures such a s a partial or total cystectomy may be required. ~::~ Laura E. Crocitto, M.~'!} Department of Urolo~) University of Southern Califori,iai 2025 Zonal Avemie:, Los Angeles, Califo
2. Cohen AS: Amyloidosis.N EnglJMed 277: 522"53ai 1967. ~i 3. Nakajima K, Hisazumi H, Okasyo A, Murayama t(, an'd:{ Matsubara F: Primary localized amyloidosis of the btaddei}~. Urology 15: 302-303, 1980 . . . . . , 4. Farah RN, Benson DO, Fine G, and Dorman PJ: Pri~: mary localized amyloidosis of bladder. Urology 13: 200-2CI}:,'i 1979. 5. Grainger R, O'Riordan B, Cullen A, KeIiy D, ali't~ Heaney J: Primary amyloidosis of lower urinary tract, t,rd{ ~ ogy 31: 14, 1988. "?' 6. Malek RS, Greene LF, and Farrow GM: Amyloidosis 6~i the urinary bladder. BrJ Urol 43: 189-200, 1971. , :.ii: 7. Kyle RA, and Greipp PR: Amyloidosis (AL). Cliniea!i and laboratory features in 229 cases. Mayo Clin Proc 58!i~ 665-683, 1983. ' .... 8. Gertz MA, Kyle RA, and Greipp PR: Response rates an~d~j survival in primary systemic amyloidosis. Blood 77: 257-26~ i 1991. -;~j 9. Gertz MA, and Kyle RA: Primary systemic amylot&-/: osis--a diagnostic primer. Mayo Clin Proc 64: 1505-1519," 1989. 10. Kyle RA, Greipp PR, Garton JP, and Gertz MA: P ~ mary systemic amyloidosis. Comparison of melphalan/preti;~ nisone versus colchicine. AmJ Med 79: 708-716, 1985 ..... ':. 11. Cohen AS: Amyloidosis, in Wilson JD, et al. (Ed;i i} Harrison's Principles of Internal Medicine, 12th ed. New Yofkl McGraw-Hill, 1991, pp 1417-1421. 12. Diseases of immunity, in Cotran RS and Kumar'~. (Eds): Robbin's Pathologic Basis of Disease 4'th ed Ph la~ieir'i phia, WB Saunders, 1989, pp 163-237.

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