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.     

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.     

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 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.     

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 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.     

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.     

Rheumatoid arthritis-induced pseudotumoral AA amyloidosis of the bladder with vesico-peritoneal fistula

Rheumatoid arthritis-induced AA amyloidosis of the bladder is rare, with fewer than 25 cases reported so far. This localization may be life-threatening with a mortality rate of about 60%, most often due to massive hematuria or multiorgan failure as a result of systemic amyloidosis. We report the case of a 72-year-old woman with a long history of rheumatoid arthritis who developed gross hematuria that induced severe anemia. Ultrasonography and tomodensitometry revealed a large mass localized in the upper part of the bladder. Cystoscopy showed a congestive inflammatory area with a large vesicoperitoneal fistula. Biopsies revealed amyloidosis, and immunohistochemical staining of the specimens defined the process as AA amyloidosis. The amyloid deposits were also found in the rectum, duodenum, uterus and kidneys. This case of rheumatoid arthritis-induced AA amyloidosis of the bladder is characterized by its pseudotumoral aspect and the existence ofa vesico-peritoneal fistula: only 2 cases have been reported so far. Treatment was symptomatic, and the patient died from cachexia. The pseudotumoral forms of AA amyloidosis, including amyloidosis of the bladder, deserve an early correct diagnosis. Otherwise, an incorrect diagnosis, especially cancer, may prompt inappropriate treatments.     

Localized amyloidosis of urinary bladder: a case report

A case of localized amyloidosis of the urinary bladder is reported. A 82-year-old woman visited our hospital with the chief complaint of miction pain and residual urine sensation. Cystoscopic examination revealed a broad-based and nonpapillary tumor without bleeding on the right lateral wall. A transurethral biopsy of this tumor was performed. A histopathological examination with H.E. and Congo red stains demonstrated amyloid deposition in the submucosal layers of the vesical wall. Rectal biopsy and other findings suggested no deposition of amyloid in other organs. On the basis of these findings, we made a diagnosis of localized amyloidosis of the urinary bladder. To the best of our knowledge, the present case is the 23rd of localized amyloidosis of urinary bladder in Japan. The patient was asymptomatic after biopsy. We discuss the clinical features and management of 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.     

Sinonasal Globular Amyloidosis Simulating Malignancy: A Rare Presentation

Primary localized amyloidosis in the head and neck region is a rare entity. The most commonly involved organ is larynx. Primary amyloidosis localized to the sinonasal tract is extremely rare. We report one such case along with a brief review of the associated literature. The aim of reporting this case is to emphasize the fact that sometimes nasal amyloidosis can also present with signs and symptoms of nasal and nasopharyngeal malignancy. The definitive diagnosis in such cases depends upon histopathology and further confirmed by immunohistochemistry. A 55-year old male presented with recurrent episodes of nasal bleed, bilateral nasal obstruction, and bilateral hearing loss from last 7 years. On clinical examination a mass was found in the nasal cavity on both sides reaching up to the nasopharynx. Contrast enhanced CT scan revealed that the mass was extending up to the skull base and destroying bony landmarks of the nasal cavity and paranasal sinuses. Mass was proved to be amyloidosis after histopathological examination. It showed multiple blotches of globular submucosal deposit of amyloid, on staining with Congo red. Immunohistochemistry confirmed AL amyloidosis with expression of mixed kappa and lambda light chain immunoglobulin (κ > λ). No evidence of systemic amyloidosis was found after proper work up. It was managed by conservative surgery.     

Primary Localized Amyloidosis of the Small Intestine Presenting as an Intestinal Pseudo-obstruction: Report of a Case

A 47-year-old man with primary amyloidosis confined to the small intestine is reported. Thickening of the folds and multiple polypoid protrusions were found in the duodenum by upper gastrointestinal endoscopy. Because the patient presented with a persistent intestinal pseudo-obstruction, partial jejunectomy was performed. Histological examination of the resected tissue revealed massive deposits of amyloid throughout the jejunal wall. Neither a predisposing condition nor any other sites of deposition were found, and primary amyloidosis of the small intestine was diagnosed. This rare form of amyloid deposition should be recognized so that an early diagnosis can be made.     

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.     

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.     

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.     

Amyloidosis of the seminal vesicle--a case report and review of the literature

The finding of amyloid deposits in the seminal vesicles has been known for many years. The deposits are usually localized and asymptomatic. In recent years seminal vesicle amyloidosis has been reported to simulate prostate and bladder cancer invasion on MRI. We therefore feel that knowledge of the entity is important and present herein a typical case confirming the previous findings that amyloidosis of the seminal vesicles is a unique form of amyloidosis, a relatively common incidental finding and one that may be related to prostate cancer.     

Case of nephrotic syndrome due to AL-type primary amyloidosis associated with renal cell carcinoma

An 80-year-old man was admitted because of appetite loss, mild proteinuria, and leg edema. A computed tomography examination revealed a tumor in his left kidney, and a left nephrectomy was performed. The tumor was histologically diagnosed as a clear cell type renal cell carcinoma, and hematoxylin eosin staining of the non-tumor region of the resected kidney showed an almost normal morphology. Three months later, he was readmitted because of the development of nephrotic syndrome with a urinary protein excretion of 4.2 g/day, a serum total protein concentration of 5.0 g/dL, a serum albumin concentration of 2.4 g/dL, a serum total cholesterol concentration of 214 mg/dL, and generalized edema. A full examination revealed no evidence of metastasis or recurrence of the renal cell carcinoma or any other malignant tumor. Congo red staining and immunohistochemical staining were performed using the non-tumor region of his resected kidney, and the presence of amyloid deposits in the microvascular walls and glomeruli that did not disappear when treated with potassium permanganate was disclosed. In this manner, the patient was diagnosed as having AL-type primary amyloidosis. Bence-Jones proteinuria and gastric amyloidosis were also observed, but a bone marrow examination showed no signs of multiple myeloma. Previous studies have reported an association between renal cell carcinoma and renal amyloidosis, mainly AA-type secondary amyloidosis. To our knowledge, only two cases of renal cell carcinoma associated with primary amyloidosis have been previously reported. Therefore, the present patient not only represents a rare case of renal cell carcinoma associated with primary amyloidosis, but also reminds us that careful histological examination of the non-tumor region of the resected kidney is needed to evaluate the proteinuria associated with renal cell carcinoma, particularly in elderly patients.     

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。     

Secondary vesical amyloidosis

The bladder involvement is a very unusual fact in systemic amyloidosis. The distinction of primary and systemic amyloidosis disease with bladder involvement (secondary bladder amyloidosis) is important to the urologist. Secondary amyloidosis of the bladder is a rare disease entity (approximately 20 cases published). We document a case of a woman with a large history of rheumatoid arthritis who developed severe macrohematuria. Diagnosis was done by biopsy that revealed amyloidosis, and it was confirmed with an immunohistochemical staining of the specimens that defined the process as amyloid AA (secondary amyloidosis).