Primary localized amyloidosis of the bladder: a case of AL (lambda) amyloid protein and combination therapy using dimethyl sulfoxide and cepharanthin.

We report a case of primary localized amyloidosis of the bladder with amyloid deposits which was characterized as being of immunoglobulin light chain origin (AL) including lambda type (A lambda) and P component (AP) using the KMnO4 pretreatment method and immunohistochemical procedures. The patient was treated successfully with intravesical dimethyl sulfoxide instillation and oral administration of high-dose cepharanthin after transurethral resection. Combination therapy with dimethyl sulfoxide and cepharanthin was shown to be useful for primary localized amyloidosis of the bladder.     

原发性膀胱淀粉样变(附4例报告)

目的：探讨原发性膀胱淀粉样变的诊治方法。方法：回顾性分析4例原发性膀胱淀粉样变的临床资料。结果：3例行电切或开放手术后辅以二甲基亚砜(DMSO)膀胱灌注，随访3～6年未见复发。1例单纯DMSO膀胱灌注，2年后复发。结论：原发性膀胱淀粉样变极易与膀胱癌混淆，需经病理及特殊染色确诊。治疗上尽可能切除病变组织，术后辅以DMSO膀胱灌注。     

原发性膀胱淀粉样变1例报告并文献复习

目的 探讨原发性膀胱淀粉样变的临床、影像学、病理学特征以及诊疗方法。方法：回顾性分析1例原发性膀胱淀粉样变患者的临床资料。结果：行经尿道电切（TUR）术,术后辅以50％二甲基亚砜（DMSO）膀胱灌注,随访1年未出现肉眼血尿。结论：原发性膀胱淀粉样变临床及其罕见,极易与膀胱癌混淆,确诊需依据临床表现、影像学检查、病理学检查及特殊染色。治疗以手术尽可能切除病变组织为主,术后辅以DMSO膀胱灌注,疗效及预后良好。     

Intravesical dimethyl sulfoxide for primary amyloidosis of the bladder

Primary bladder amyloidosis is a rare disease. Treatment recommendations are necessarily anecdotal. We report a case of a 52-year-old woman treated successfully with intravesical dimethyl sulfoxide instillation.     

Localized amyloidosis of the urinary bladder: a case report

Amyloidosis is characterized by extracellular deposition of abnormal insoluble fibrils, which cause structural and functional disorders. Amyloidosis is classified into systemic and localized amyloidosis. Localized amyloidosis in individual organs is uncommon. We report a rare case of localized form of primary amyloidosis of the urinary bladder. A 76-year-old male visited our hospital with a complaint of macroscopic hematuria. Cystoscopy showed submucosal hematoma in the anterior wall and broad-based mass occupying the trigone without normal mucosa covered by calcification. Transurethral biopsy and resection were performed. Histopathological diagnosis was AL type amyloidosis occupying submucosal extracellular space. We gave the patient occlusive dressing with dimethyl sulfoxide. In 12 months, cystoscopy and magnetic resonance imaging revealed improvement of the mass-like lesion in the bladder wall.     

Intravesical dimethyl sulfoxide instillations in the treatment of secondary amyloidosis of the bladder

A-22-year-old woman with long-standing rheumatoid arthritis and secondary amyloidosis of the bladder had recurrent profuse macroscopic hematuria. She was treated with intravesical dimethyl sulfoxide instillation every 2 weeks for 1 year. She remained asymptomatic during the treatment and at 6 months. Progressive disappearance of amyloid from the superficial mucosa of the bladder was demonstrated in sequential histological examinations.     

Primary localized amyloidosis of the bladder: experience with dimethyl sulfoxide therapy

PURPOSE: We present our long-term experience with intravesical dimethyl sulfoxide (DMSO) for primary localized amyloidosis of the bladder. MATERIALS AND METHODS: The study included 4 males and 2 females 28 to 68 years old (mean age 54) at diagnosis of biopsy proven primary localized amyloidosis involving the bladder diffusely or extensively in 1 locale. All patients had normal upper urinary tracts. They continued to be symptomatic (hematuria in 3, irritative voiding symptoms in 1, and hematuria and irritative voiding symptoms in 2) despite conventional transurethral destructive therapy. Every 2 weeks they received 30-minute instillations of 50 ml. 50% DMSO intravesically for 3 months (patient 1), 6 months (1) and 1 year (4). RESULTS: Therapy failed at 3 and 6 months in 2 patients of whom 1 with a contracted bladder underwent cystectomy and another was stabilized for 1 year with laser therapy. In the remaining 4 patients who were followed for 6 years disease stabilized for 2 to 6 years (mean 3.5) but 3 later required additional therapy including repeat DMSO in 1 and laser therapy in 2. CONCLUSIONS: Diffuse or locally extensive bladder involvement by primary localized amyloidosis usually fails to respond to conventional transurethral destructive surgical procedures. Collectively, our experience and the literature suggest that intravesical DMSO can be a bladder saving measure and help resolve ureterovesical obstruction in some patients. High recurrence rate mandates lifelong cystoscopic surveillance.     

Localized amyloidosis of the ureter and bladder treated effectively by occlusive dressing technique therapy using dimethyl sulfoxide: a case report

We report here the first case of localized amyloidosis of the ureter and bladder to be treated effectively by occlusive dressing technique therapy using dimethyl sulfoxide. The patient was a 48-year-old woman whose chief complaint was macrohematuria and right back pain. Ultrasound sonography demonstrated right hydronephrosis and an intravesical mass in the region of the right ureteral orifice. Retrograde pyelography revealed severe stricture of the right lower ureter. Cystoscopy demonstrated a yellow submucosal tumor around the right ureteral orifice. We suspected urinary tract amyloidosis, and transurethral biopsy and resection of the intravesical mass were performed under right ureteral stenting. Histopathological diagnosis was amyloidosis. There was no evidence of systemic amyloidosis. To treat residual amyloidosis of the ureter and bladder, we performed occlusive dressing technique therapy using dimethyl sulfoxide every day. After 6 months of therapy, the right hydronephrosis disappeared, and there was no evidence of a recurrence of amyloidosis. We concluded that this therapy was very effective and safe for urinary tract amyloidosis.     

A case of bladder amyloidosis

Herein we report a case of bladder amyloidosis treated successfully with TUR & DMSO bladder instillation. A diagnosis was made by a biopsy of the bladder epithelium. Amyloid fibrils were confirmed in the biopsy specimen with polarization and electron-microscopy. The patient was treated with TUR. The residual lesion had disappeared with DMSO bladder instillations for 4 months (12X) without side effects after TUR. Thus DMSO bladder instillation with surgical resection seems to be an excellent therapy for bladder amyloidosis.     

A case of localized amyloidosis of the bladder manifesting post-renal failure

We report a case of primary localized amyloidosis of the bladder which manifested post-renal failure. A 79-year-old woman with diabetes mellitus complained of anorexia and oliguria. Computed tomographic (CT) scan showed bilateral hydronephrosis. Cystoscopic examination revealed a broad-based nonpapillary tumor in the trigonum of the bladder and CT scan demonstrated thickening of the posterior wall of the bladder. Pathological examination of the transurethral biopsy specimen revealed amyloid deposits in the submucosa, but no malignant changes were found. Cytodiagnosis of washing fluid of the bladder revealed amyloid deposits around the exfoliative cells. Serum electrophoresis showed a normal pattern. Urinary Bence-Jones protein was not detected. Amyloid deposits were not found in rectal mucosa. Systemic or secondary amyloidosis was ruled out from these findings, and primary localized amyloidosis of the bladder was diagnosed. The mass of the bladder was transurethrally resected and pig-tail stents were indwelt. These procedures gave a satisfactory result.     

Solitary primary amyloidosis of urinary bladder Light and electron microscopic study

A fifty four-year-old white woman with a history of episodic gross hematuria was diagnosed as having solitary primary amyloidosis of the urinary bladder involving a large segment of the dome and anterior wall. A segmental resection of the bladder was performed with good results. Electron microscopic examination of the lesion revealed evidence to indicate that amyloid in this case was produced by the fibroblasts within the lamina propria of the bladder. This mode of amyloidogenesis is identical to that noted in some of the previously published electron microscopic studies on localized amyloidosis of skin. Solitary primary amyloidosis of the urinary bladder is a rare lesion with only 46 cases having been previously reported in the literature.     

Primary localized amiloidosis with recurrent relapse: a case report]

This is a case report of primary localized amyloidosis of the urinary bladder. The patient was a 73-year-old woman who had relapse of this disease three times. Primary localized amyloidosis of the urinary bladder is an especially rare disease and the prognosis is excellent in most cases. We performed transurethral resection of the bladder tumor (TUR-Bt) each time and we are following up this patient carefully. After the last TUR-Bt, she has had no recurrence for 7 months. Considering multifocal recurrence and excellent prognosis, we stress the importance of TUR-Bt for diagnosis and careful follow up.     

Primary amyloidosis of lower urinary tract

Primary amyloidosis of the lower urinary tract is a rare condition with an excellent prognosis in most cases. Three patients with this condition are described. In the cases of localized amyloidosis of the urethra and urinary bladder, the clinical presentation mimicked cancer of the respective sites. This was also true in the case of primary systemic amyloidosis involving the bladder. If significant associated systemic or local disease can be excluded, management is symptomatic and expectant.     

Report of 2 cases of isolated, primary amyloidosis of the male urethra and urinary bladder of a female

Primary localized amyloidosis of the bladder and urethra is a rare condition. Two cases of isolated primary amyloidosis, of the urethra in a man and of the bladder in a woman, are reported. The clinical specificity of the location and the specific therapy are discussed. We draw attention to the extended, palette of diagnostic investigations now available and the new classification made possible by the introduction of immunohistochemistry and immunoelectron microscopy.     

Primary localized amyloidosis of the bladder: an immunohistochemical study of a case.

We report a case of primary localized amyloidosis of the bladder causing renal failure. Immunohistochemically, amyloid fibril protein originated from the lambda type light chain of immunoglobulin.     

Primary localized AA type amyloidosis of urinary bladder: case report of rare cause of episodic painless hematuria

A 45-year-old diabetic man presented with an episodic history of painless gross hematuria. He had had no previous urinary symptoms or any other medical problems. His physical examination, urine cytology, and computed tomography scan findings were normal. Cystoscopy demonstrated two nodular masses in the urinary bladder with a hemorrhagic mucosal surface. Biopsies revealed amyloidosis, and immunohistochemical staining of the specimens defined the process as amyloid AA (mostly seen in secondary amyloidosis). The workup for systemic conditions associated with amyloid AA was negative. This represents an unusual case of primary localized AA-type amyloidosis of the bladder.     

Current Recommendations for Bladder Instillation Therapy in the Treatment of Interstitial Cystitis/Bladder Pain Syndrome

Bladder instillation therapy refers to the direct introduction of medication into the bladder and is a common treatment modality for patients with interstitial cystitis/bladder pain syndrome (IC/BPS) who have failed conservative and oral therapies. The current American Urological Association (AUA) recommendations list three medications as options for IC/BPS instillation therapy: dimethyl sulfoxide, heparin, and lidocaine. The purpose of this review is to examine the evidence behind the recommendations for these medications. We also examine several historical or experimental therapies that do not hold recommendations but are still used on rare occasion. Finally, we discuss our bladder instillation strategies as well as potential future research and development in intravesicular therapy.     

Primary localized amyloidosis of the urinary bladder: a case report

A case of primary localized amyloidosis of the bladder during a twelve-year period is described. The initial symptom was miction pain when she was 43 years old. Cystoscopic examination revealed yellowish elevated lesion with hemorrhage at the right side of the bladder neck. Transurethral resection was performed, but two years later recurrence was found at the trigonum as well as the original site. The two lesions were apart. She remained asymptomatic for the following ten years until she returned to us with bladder symptoms. Cystoscopy showed the lesion was more diffuse and the posterior wall was involved in continuity with the trigonum. Complete transurethral resection was not possible because of generalized bladder involvement. Our case illustrates the clinically benign but slowly progressive nature of amyloidosis of the bladder. Multifocal recurrence is also to be noted. Our current treatment policy is conservative treatment with careful follow-up. She is asymptomatic three months postoperatively.     

The use of dimethyl sulfoxide in the treatment of intractable urinary frequency

Intravesical instillation of dimethyl sulfoxide (DMSO) was used in the treatment of patients with intractable urinary frequency due to chronic prostatitis, chronic cystitis, tuberculous contracted bladder and interstitial cystitis. Before the application of this therapy, all 4 patients were examined carefully to rule out cases of acute infectious diseases of the urinary tract, active urinary tuberculosis, neurogenic bladder and carcinoma in situ of the bladder. Three of the four patients achieved an excellent response both subjectively and objectively. In the United States, intravesical instillation of DMSO had already been established as the specific method in the treatment of interstitial cystitis and no side effects have been reported so far. Therefore, we recommend the use of intravesical instillation of DMSO more commonly in various forms of intractable urinary frequency.     

Eosinophilic cystitis after bladder instillation with dimethyl sulfoxide

Eosinophilic cystitis is a rare and poorly understood disorder. We report the first case of an acute flare of eosinophilic cystitis in a 51-year-old woman after bladder instillation with dimethyl sulfoxide (DMSO) for presumed interstitial cystitis. The patient presented with severe bladder pain, fever, and eosinophilia several hours after instillation. These symptoms were unresponsive to conventional analgesic and antibiotic treatments. Cystoscopy revealed erythema and exudate at the bladder walls, along with edema of both ureteral orifices. Bladder biopsies demonstrated massive eosinophilic infiltration of the bladder, confirming the diagnosis of eosinophilic cystitis. Urologists should bear in mind this clinical entity, particularly when DMSO is administered to patients with multiple drug allergies.