Amyloidosis Of Bladder Research Articles

Localized Urinary Bladder Amyloidosis as Urothelial Cancer Mimicker.

Localized amyloidosis of the bladder is rare and often mimics bladder malignancy. It is typically associated with the extracellular deposition of monoclonal light chains, either κ or λ. The cause is unknown, but it is thought to be due to chronic inflammation/cystitis. To highlight the importance of localized urinary bladder amyloidosis as a rare mimicker of urothelial malignancy and elucidate its clinical, histopathologic, and cytopathologic manifestations. Cases of urinary bladder amyloidosis diagnosed during 2000-2023 were retrieved retrospectively from pathology archives. Electronic medical records, including cystoscopy findings and pathology slides including Congo red stain, were reviewed. Here we present 6 patients with localized urinary bladder amyloidosis. Four of the 6 patients were women, with ages ranging from 46 to 69 years, and a mean age of 58 years. Five of 6 patients presented with hematuria, while in 1 patient, bladder amyloidosis was discovered incidentally. Cystoscopy findings invariably were concerning for malignancy, with raised erythema in 5 patients and fungating mass protruding into the bladder lumen in 1 patient. Bladder biopsies and urine cytology were negative for malignancy in all cases. Congo red-positive amyloid deposits involved lamina propria with sparing of the detrusor muscle. In 5 cases, the deposits were typed as derived from the λ light chain, whereas no information was available for 1 patient. Subsequent clinical workup ruled out systemic amyloidosis. These cases of urinary bladder amyloidosis highlight the importance of considering rare amyloidosis in the differential diagnosis of hematuria and cystoscopy with a lesion mimicking malignancy.

A Rare Clinical Condition That Can Mimic Bladder Malignancy: Primary Bladder Amyloidosis

A Rare Clinical Condition That Can Mimic Bladder Malignancy: Primary Bladder Amyloidosis

Diagnostic Dilemma in Primary Localized Bladder Amyloidosis (PLBA): an Experience from a Tertiary Care Centre

Diagnostic Dilemma in Primary Localized Bladder Amyloidosis (PLBA): an Experience from a Tertiary Care Centre

Urinary bladder amyloidosis presenting as recurrent cystitis

Urinary bladder amyloidosis is rare. Our patient had a history of repeated lower urinary tract symptoms for the last 2 years, for which she was treated as urinary tract infection. Ultrasonography showed thick urinary bladder wall. She underwent cystoscopy and biopsy was performed. Immunostaining of the biopsy material with Congo red stain showed the presence of amyloid fibrils within the biopsy. Amyloid A (AA) staining was negative. Immunohistochemistry (IHC) showed faint Kappa band positivity along amyloid deposits. Serum-free light chain assay and serum protein electrophoresis report were normal. Thus, we report a rare case of urinary bladder limited amyloidosis who presented with frequent lower urinary tract symptoms.

Bladder Amyloidosis as a Rare Cause for Hematuria

Bladder Amyloidosis as a Rare Cause for Hematuria

PRIMARY BLADDER AMYLOIDOSIS - A GREAT MIMICKER OF UROTHELIAL MALIGNANCY.

Primary bladder amyloidosis is a rare condition that often mimics bladder malignancy. Here we report a case of primary bladder amyloidosis presenting as gross hematuria with clots which was managed by cystoscopy and fulguration. It is a diagnosis of exclusion usually revealed in the histopathological examination and is a relatively benign disease with better outcomes.

A Rare Cause of Lower Abdomen Pain – Primary Urinary Bladder Amyloidosis

Primary urinary bladder amyloidosis is a rare disease. Its importance lies in the ability of the disease to masquerade the carcinoma urinary bladder in clinical presentation and imaging but with a distinctively contrasting treatment. The commonest presentation is painless hematuria. Definitive diagnosis is made on histopathology and by ruling out systemic amyloidosis. Here, we report a 60-year-old male presenting with lower abdomen pain diagnosed with primary urinary bladder amyloidosis and discuss the management based on current literature.

Plasma cell neoplasms of the bladder: a series of 9 cases

From 2009 to 2017, we identified 9 cases of plasma cell neoplasms on biopsies of the bladder in patients without a history of plasma cell myeloma or transplantation (6 men and 3 women). Four of the nine showed amyloid deposition, of which one additionally revealed a clear cell adenocarcinoma of the bladder. Follow-up was obtained in 7 cases. Of 3 cases (including 2 with amyloid) for which electrophoresis and immunofixation results were obtained, the 2 amyloid cases showed evidence of serum or urine paraproteins: serum IgM kappa in a patient with kappa light chain–restricted plasma cell neoplasm and urine IgA lambda in a patient with lambda light chain–restricted plasma cell neoplasm. By way of contrast, 1 case with kappa light chain–restricted plasma cell neoplasm in the absence of amyloid showed no serum monoclonal protein. Bone marrow biopsy results were obtained in the 2 amyloid cases revealing a population of 5% or less plasma cells with no assessment of clonality and, thus, were not diagnostic of plasma cell myeloma. In congruence, the 2 amyloid cases also showed no radiologic evidence of systemic plasma cell myeloma. One patient with plasma cell neoplasm only received chemotherapy and radiation without subsequent biopsies; one patient with plasma cell neoplasm, amyloid, and clear cell adenocarcinoma received radiation with an absence of neoplastic disease on subsequent biopsies. In addition, no evidence of systemic amyloid was found in the cases with bladder amyloidosis. Plasma cell neoplasms of the bladder, with and without amyloid deposition, are rare; this is the first known case series. In 7 cases with follow-up, plasma cell myeloma did not appear to manifest in a 1- to 127-month follow-up. However, paraproteins were identified on further testing in 2 cases with amyloid. Although bladder plasma cell neoplasms with and without amyloid tend to have a favorable prognosis in short-term follow-up, our study supports the need for additional workup for systemic disease, particularly in those with concurrent amyloidosis.

Long Term Follow Up of Bladder Amyloidosis

This is an evaluation of a patient presenting with bladder amyloidosis and a seven year asymptomatic history between gross hematuria episodes. Cystoscopic evaluation showed a diffuse progression of amyloid deposits throughout the bladder. Biopsy confirmed the diagnosis and was negative for malignancy. Further evaluation ruled out systemic disease. To date, he has declined treatment. There is little available literature on long term urologic follow up for these patients. Bladder amyloidosis is rare, but most urologists will encounter it in their clinical practice. There are no agreed upon guidelines or recommendations for long term management of these patients, likely owing to its rarity. This patient had local progression confirmed on biopsy without evidence of malignancy. Evaluation for systemic amyloidosis was also negative. While a single, isolated case, it is unique for the length of follow up, the local progression and lack of symptoms. It is consistent with the low likelihood of systemic progression but does raise the question of how to treat these patients long-term. The authors recommend initial biopsy both to confirm diagnosis and rule out malignancy, evaluation for systemic disease and regular cystoscopic examinations to help direct therapy.

Amyloidoza pęcherza moczowego — opis przypadku i przegląd piśmiennictwa

Amyloidosis is a heterogeneous group of protein misfolding diseases caused by extracellular deposition of abnormal beta-fibrils resistant to proteolysis. The most common type is light-chain amyloidosis (AL amyloidosis) which can be systemic or localized. Localized amyloidosis mostly affects the respiratory airways, genitourinary tract, gastrointestinal system or skin. We present diagnostic and therapeutic approach in a 25-year-old female patient diagnosed with urinary bladder amyloidosis. The main complaint was macroscopic hematuria occurring periodically for 2 years. Abdominal and pelvic computed tomography revealed single 25-mm-thick soft-tissue lesion of the left bladder wall. Histopathological examination of the lesion biopsied during transurethral resection of bladder tumor showed amyloid deposits with strong positive immunostaining for transthyretin, weaker for light chain (AL) and weak for serum amyloid A (AA). Serum protein electrophoresis and immunofixation did not reveal monoclonal protein. X-rays of flat bones presented without lytic lesions. There were no amyloid deposits both in trephine biopsy and subcutaneous fat biopsy. Primary systemic AL amyloidosis was excluded. According to the results of (99m)Tc-DPD scintigraphy and genetic analysis of transthyretin gene (TTR), the ATTRm amyloidosis was also excluded. Consultative histopathological analysis of the bladder biopsy made in the National Amyloidosis Center in London revealed amyloid deposits stained to lambda light chains, confirming the diagnosis of localized AL lambda bladder amyloidosis. The patient was qualified for surgical treatment. Partial resection of the bladder wall with no need for left ureter transplantation was performed. Primary localized bladder amyloidosis is a very rare entity. In the diagnostic approach the most important is the exclusion of primary systemic amyloidosis due to a completely different treatment method.

Radiologic Findings of Primary Localized Amyloidosis of Urinary Bladder

Radiologic Findings of Primary Localized Amyloidosis of Urinary Bladder

PRIMARY LOCALIZED AL AMYLOIDOSIS OF THE BLADDER MIMICKING RECURRENCE OF BLADDER CARCINOMA

Primary bladder amyloidosis is a rare disease, with approximately 200 cases documented in the literature. We herein present a 85-year-old Japanese man who has undergone a transurethral resection of a bladder tumor (TURBT) and has regularly been followed up after surgery. Since cystoscopy revealed mucosal irregularity, he has got a TURBT again for a suspicion of recurrence. There were no malignant findings in pathological diagnosis and we diagnosed as amyloidosis because it showed positive by Congo-red staining. We added immunohistological diagnosis to diagnose as localized AL amyloidosis of the bladder finally.

Images in urology: Localized primary amyloidosis of the urinary bladder.

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External beam radiation therapy for amyloidosis of the urinary bladder

Amyloidosis is a condition characterized by the extracellular deposition of amyloid, a fibrillary substance consisting of low-molecular-weight protein subunits. Amyloidosis may present either as a systemic disorder or as a process isolated to a specific organ without systemic involvement. [1] Abbas AK Diseases of immunity. in: Kumar V Abbas AK Fausto N Robbins and Cotran's Pathologic Basis of Disease. 7th ed. Elsevier Saunders, Philadelphia, PA2005: 258-264 Google Scholar

Cases of a Borderline Pathology That Can Mimic Bladder Cancer: Primary Amyloidosis of Urinary Bladder

Cases of a Borderline Pathology That Can Mimic Bladder Cancer: Primary Amyloidosis of Urinary Bladder

Primary Bladder Amyloidosis Mimicking Bladder Malignancy: A Case Report and Literature Review

Introduction and Objectives: We report a rare case of localized primary bladder amyloidosis, and we conducted a literature review. Materials and Methods: A 70-year-old man with gross haematuria and storage bladder symptoms was diagnosed with primary bladder amyloidosis with clinical and radiological features of bladder tumor. A comprehensive review of English literature was done. PubMed, NCBI and Science Direct were searched using the following search terms: primary; Bladder; Amyloidosis. Results: Primary localized bladder amyloidosis is a rare and benign urological disease with approximately 210 reported cases in the English literature. It is usually of the AL type and commonly presents with gross painless haematuria and storage bladder symptoms, mimicking bladder malignancy. Imaging investigations such as ultrasound scan, CT scan and MRI are not diagnostic, whereas histopathological examination confirms the diagnosis. Transurethral resection of the bladder lesion is the treatment of choice; however local recurrence is common (50% of cases).

Primary amyloidosis of the bladder mimicking bladder tumor

Amyloidosis is a benign, non-neoplastic disease characterized by the deposition of extracellular fibrillar protein in tissues or organs, which may be systemic or localized. Primary amyloidosis localized in the bladder is very rare in the literature and may mimic bladder cancer at presentation. A 31-year-old female patient consulted to our clinic and was diagnosed with primary bladder amyloidosis; intravesical dimethyl sulfoxide (DMSO) therapy was started. To the best of our knowledge, this patient represents the fourth case in the Turkish medical literature.

Primary Localized Amyloidosis of Urinary Bladder: A Case Report and Review of Literature

Amyloidosis is a disorder of protein metabolism characterized by extracellular deposition of abnormal protein fibrils. It may either be localized to any organ or systematically distributed throughout the body. The biochemical nature of proteins varies but the physical and tinctorial properties are shared by all the amyloidogenic proteins. In the West, it is mainly composed of amyloid light (AL) type immunoglobulin (Ig) light chains. Amyloidosis of the genitourinary tract is rare except for the kidney and isolated primary amyloidosis of the urinary bladder is even rarer. It mainly presents as intermittent painless gross hematuria. It mimics transitional cell carcinoma on imaging and endoscopic examination. We herein present a case of fifty six-years-old male with history of painless hematuria for three months. Cystoscopy revealed a 1 cm hyperemic area on the posterior wall of urinary bladder. The biopsy showed features of amyloidosis and amyloid A (AA) immunostaining was negative. Extensive workup was done to exclude other sites of involvement and a final diagnosis of primary localized amyloidosis of the urinary bladder was made. The patient is on regular follow-up.

Occlusive dressing therapy using dimethyl sulfoxide in a patient presenting with primary localized amyloidosis of the urinary bladder: a case report.

IntroductionAmyloidosis is characterized by extracellular deposition of abnormal insoluble fibrils, which cause structural and functional disorders. Amyloidosis is classified into primary and secondary disease. We report a case of localized amyloidosis of the urinary bladder. In the English literature, this is the first case effectively treated with occlusive dressing therapy using dimethyl sulfoxide.Case presentationA 58-year-old Japanese woman was introduced to our department with asymptomatic gross hematuria. Cystoscopy revealed a gently raised nodule at the right lateral wall. Histopathological findings of this lesion revealed extensive amorphous eosinophilic deposits that stained positive with Congo red and Dylon. The patient was diagnosed with primary localized amyloidosis of the urinary bladder. To treat residual amyloidosis of the bladder, we performed occlusive dressing therapy using dimethyl sulfoxide. After treatment, cystoscopy and magnetic resonance imaging showed no relapse of the mass-like lesion of the bladder wall.ConclusionsOcclusive dressing therapy using dimethyl sulfoxide is efficacious and tolerable for amyloidosis of the urinary bladder. The maneuver of occlusive dressing therapy was simpler and easier than that of intravesical instillation, and occlusive dressing therapy was advantageous in that the patient could perform the therapy herself every day. However, invasive surgical management including cystectomy should be considered if conservative management is inefficacious.

Urinary Cytopathology in Primary Bladder Amyloidosis

Background: Amyloidosis results from the accumulation of unique extracellular proteins which are not able to be degraded via the usual mechanism of lysosomal proteolysis. Isolated collections of amyloid within the bladder are extremely uncommon, and a cytopathologic description in voided urine has not been described to date. Methods: A retrospective review was performed at a tertiary-care hospital, and 3 patients with isolated bladder amyloidosis and corresponding voided urine specimens were identified. The following clinical data were collected for each case: age, gender, treatment and follow-up information. Results: The patient age range was 76-84 years, with 2 males and 1 female. Amyloidosis was never clinically suspected. In 1 patient, a urinary amyloid manifested, which was thought to represent extraneous debris at the time of original diagnosis. Two patients never manifested signs of systemic amyloidosis or multiple myeloma, and the third was found to have a monoclonal gammopathy. Conclusions: Our results show the difficulty of diagnosing urinary amyloid in the absence of clinical suspicion. Further, the presence of urinary amyloid is unlikely in patients with bladder amyloidosis as the cohesive nature of the protein makes spontaneous shedding uncommon. Testing for systemic amyloidosis is warranted and if the disease is localized, a favorable outcome can be expected.