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.     

Primary amyloidosis of the bladder: a case report

A case of primary amyloidosis of the bladder, which developed 4 years after primary treatment, is described. The patient is a 55-year-old male, who consulted our clinic with a complaint of asymptomatic gross hematuria on October 1, 1983. Cystoscopic examination showed a tumorous lesion involving right lateral wall of the bladder, which was resected transurethrally. No malignancy was found by histopathological examination. About 4 years later, he consulted our clinic again with a complaint of asymptomatic gross hematuria. There was a tumorous lesion involving left lateral wall of the bladder on cystoscopy. Transurethral resection of bladder tumor was performed under a diagnosis of bladder tumor. Histological examination revealed amyloidosis of the bladder, AL-type amyloid. Re-examination of the specimens resected 4 years ago showed the same findings. Biopsies from the tongue, skin and rectum disclosed no amyloid deposition. During these 19 months, he had no recurrence. This case was thought to be the first one of primary amyloidosis of the bladder showing new recurrence reported in Japanese literature.     

A case of amyloidosis of the ureter caused by primary macroglobulinemia

A 78-year-old woman was referred to our hospital with chief complaint of colicky right flank pain. Computed tomography (CT) demonstrated right hydronephrosis and a wall thickening in the right ureteropelvic junction. Right nephroureterectomy was performed under a clinical diagnosis of invasive ureteral cancer. Histological examination revealed immunoglobulin light chain (AL) amyloidosis of the ureter. Although amyloid deposition seemed to be localized to the ureter alone, bone marrow biopsy revealed primary macroglobulinemia. After 23 months, no signs or symptoms suggesting the progression of amyloidosis or macroglobulinemia have been pointed out. AL amyloidosis arising secondarily to plasma cell disorder is called immunocytic amyloidosis. In general, immunocytic amyloidosis accompanies deposition of the amyloid protein to various organs of the whole body, and causes multiple clinical symptoms related to amyloid deposition. Even though the clinical manifestation indicates the localized disease like the presented case, close examination of immunocytic amyloidosis is important.     

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.     

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 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.     

Localized amyloidosis of the ureter: report of a case

A case of primary amyloidosis of the ureter is presented. The patient was a 48-year-old woman with a 5-year history of asymptomatic macrohematuria. Roentgenographic examination showed left hydronephrosis and stenosis of the left distal ureter. Histological examination of the biopsied specimen during the operation revealed no malignancy; and, left partial ureterectomy and uretero- vesiconeostomy was performed. Congo red stain proved amyloid deposition after surgery. Rectal biopsy was negative for amyloid; and, other laboratory findings were within normal range. Thus it was diagnosed as a primary localized amyloidosis of the ureter. Importance of intraoperative histologic examination was discussed. This is the 22nd case in the literature.     

Localized primary (AL) amyloid tumor of the breast. Cytologic, histologic, immunocytochemical and ultrastructural observations

Two examples of localized primary amyloid tumor of the breast are presented, including one patient with metachronous bilateral lesions. Our findings and review of the literature indicate that this rare lesion occurs predominantly in elderly females and can be mammographically and clinically confused with carcinoma. Fine-needle aspiration biopsy can be a useful procedure to make a preliminary diagnosis. Congo red staining with prior potassium permanganate incubation confirmed the AL type of amyloid in our two cases; this might be the predominant type in the localized form involving the breast. Immunofluorescence studies demonstrated IgA, with kappa and lambda light-chain deposition within the amyloid foci in one case, and intracytoplasmic IgG with both light chains within plasma cells and amyloid deposits of the second case. Ultrastructural examination of one of the cases showed characteristic findings of straight, nonbranching fibrils of 4-9 nm, diagnostic of amyloid. From our findings and a review of the literature, we conclude that amyloid tumors of the breast can occur in three separate settings: secondary amyloidosis, systemic or multiple myeloma associated amyloidosis, and as a localized primary type having a benign course.     

Amyloidoma of the chest wall: a rare entity

Amyloidoma (tumoral amyloidosis) is defined as a solitary localized tumor-like deposit of amyloid, in the absence of systemic amyloidosis. Amyloidoma is the least common presentation of tissue amyloid deposition, reported in many anatomic sites including the respiratory, genitourinary and gastrointestinal tracts, as well as the central nervous system, skin, breast and soft tissue. Amyloidoma of the chest wall is extremely rare, and to date only one case has been reported in literature. The authors present a case of a chest wall tumor that causes local destruction, being an amyloidoma on histopathologic examination. It was treated with wide local excision, with no recurrence during almost two years of follow-up. A search for occult systemic disease is recommended and was also performed.     

A case of emphysematous cystitis with familial amyloidosis

This is a report of the thirteenth known case in Japan of emphysematous cystitis. A 70-year-old man visited our hospital because of pollakisuria and macrohematuria on November 21, 1989. The patient had been known to have familiar amyloid polyneuropathy for the previous 3 years. Urinalysis showed marked hematopyuria. The residual urine was 216 cc, and urine cultures yielded 10,000,000 colonies of Escherichia coli per ml. Laboratory studies revealed systemic inflammatory findings, but no diabetic change. A plain X-ray film of the abdomen and an excretory urogram (DIP) showed small linear and round gas collections in the bladder shadow. A CT scan revealed multiple gas locules within the bladder wall. A diagnosis of emphysematous cystitis was established. The patient was given antibiotics, and there was striking clinical improvement. Histological examination of the endoscopically obtained biopsy specimen of the bladder revealed amyloidosis. We believe that this patient had a cystitis emphysematosa precipitated neurogenic bladder due to amyloid polyneuropathy and amyloidosis of the bladder.     

Primary bladder amyloidosis

Primary amyloidosis of the bladder is a rare disease entity manifested as extracellular deposits of a fibril protein in an amyloid substance form. Clinical presentation resembles a bladder tumor, as hematuria is the most common clinical manifestation. Single organ affectation is most frequent in the bladder, however, we have found less than 100 cases. We present the case of a woman with primary amyloidosis of the urinary bladder with symtoms of hematuria. Endoscopic examination suggested a bladder tumor.     

A case of localized nodular pulmonary amyloidosis diagnosed by video-assisted thoracoscopic surgery

Pulmonary amyloidosis is a rare pulmonary lesion. We report a 42-year-old male admitted for further evaluation of abnormal chest shadow. A chest CT demonstrated a nodular shadow in right S3. We tried transbronchial and percutaneous lung biopsy, but could not diagnose. We performed partial lung resection by video-assisted thoracoscopic surgery. Pathological diagnosis of the lesion was pulmonary amyloidosis. Immunohistochemical examination revealed this amyloid composed of AL kappa protein. No clinical evidence of amyloid deposits were found in other organs. We finally diagnosed this case as a localized nodular pulmonary amyloidosis.     

Primary paraganglioma of the urinary bladder with high serum CA19-9 level: a case report

A case of primary paraganglioma of the urinary bladder with a high serum CA19-9 level is reported. A 44-year-old woman visited our hospital with the chief complaint of lower abdominal pain. Magnetic resonance imaging (MRI) examination incidentally revealed a cystic bladder tumor. Cystoscopy disclosed a broad-based non-papillary tumor on the posterior wall of the urinary bladder. With the diagnosis of a bladder submucosal cystic tumor transurethral needle puncture and biopsy were performed. The solution sampled with puncture was bloody. The patient suddenly complained of headache and blood pressure was elevated to 215/120 mmHg when we held the tumor with a cold cup biopsy forceps. Catecholamine levels of the solution in the tumor were abnormally elevated. The serum CA19-9 level was also raised. Ten days later, she underwent partial cystectomy. Histological findings of the removed specimen showed primary paraganglioma of the urinary bladder. The serum CA19-9 level decreased to normal limits on the 28th postoperative day. Our experience suggests that the level of serum CA19-9 may serve as a useful index for observing the clinical course of a patient with this disease.     

Secondary amyloidosis of the bladder causing macroscopic hematuria

Bladder involvement in amyloidosis is unusual. The case of an 80-year-old man with macroscopic hematuria caused by secondary amyloidosis of the bladder is described. Cystoscopic examination revealed only a diffuse edematous area and bleeding. No tumor-like lesions were identified. Transurethral biopsy revealed amyloid deposits. Macroscopic hematuria disappeared spontaneously after cystoscopy and bladder biopsy. The patient has been followed up without treatment and is currently free of symptoms.     

Localized amyloidosis of the bladder treated effectively by occlusive dressing technique therapy using Dimethyl sulfoxide (DMSO): two case reports

The first case is a 50-year-old female. She was consulted to our hospital for further examination of right hydronephrosis that was shown by ultrasonography. Cystoscopy revealed tumorous lesion around the right orifice. Bladder tumor was suspected and transurethral biopsy of the bladder was performed. The second case is a 54-year-old man. He was consulted to our hospital because right hydronephrosis was shown by counterized tomography. A tumorous or stenotic lesion in the lower end of ureter was suspected. Cystoscopy revealed yellowish and thickened tissue in the trigonal area. Bladder amyloidosis was suspected and transurethral biopsy of the bladder was performed. The histopathological diagnosis indicated amyloidosis and systemic amyloidosis was excluded. Both cases were treated by occlusive dressing technique therapy using Dimethyl sulfoxide (DMSO) successfully.     

Inflammatory pseudotumor of the urinary bladder: a case report

A 65-year-old female was referred to our hospital with a complaint of urinary retention and macrohematuria. Ultrasongraphy, computed tomography, magnetic resonance imaging and cystoscopy revealed a 2 x 2 cm broad-based nonpapillary bladder tumor localized in the anterior wall of the urinary bladder. The clinical diagnosis was an invasive bladder tumor and transurethral resection of bladder tumor carried out for pathological investigation. Finally, we diagnosed it as inflammatory pseudotumor of urinary bladder. No local recurrence was seen 12 months after surgery. Inflammatory pseudotumor of urinary bladder is a relatively rare condition, and this is the 52nd case reported in the Japanese literature.     

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.     

Localized Primary Amyloidosis of the Prostate,Bladder and Ureters

Primary amyloidosis of lower urinary tract is a rare entity and is usually localized to a single site. Its clinical features, and cystoscopic and radiological findings are indistinguishable from neoplastic or inflammatory lesions. We report an unusual case of amyloidosis involving multiple sites (prostatic stroma, trigone and lower ureters) in the lower urinary tract. MRI findings of bladder amyloid, which could be used to suspect this condition, are also described.     

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.     

A case of pheochromocytoma of the urinary bladder

A case of pheochromocytoma of the urinary bladder is reported. A 17-year-old male was admitted to our hospital because of gross hematuria and urinary retention on Nov. 10, 1985. There was no history of hypertension. Intravenous pyelography and cystogram demonstrated a filling defect in the bladder. Transabdominal sonography disclosed a solid mass in the anterior wall and bladder neck. Cystoscopic examination revealed a non-papillary and broad based tumor in the anterior wall and bladder neck. Partial resection of the bladder wall was performed under the diagnosis of bladder tumor. The histological diagnosis was pheochromocytoma of the urinary bladder.