Tracheobronchopathia osteoplastica: Its relationship to primary tracheobronchial amyloidosis

An uncommon case of tracheobronchopathia osteoplastica in a 52-year-old woman is reported, the fifth example of this condition to be published in the British literature. The bronchoscopic feature of bony hardness of the bronchial wall was initially suggestive of bronchial neoplasm. The histological feature of bony deposits (containing marrow showing active haemopoiesis) in the submucosa of the lower trachea and major bronchi was superimposed on a background of primary localized amyloid infiltration, and yet the bronchoscopic findings and the naked-eye morbid anatomical appearance suggested tracheobronchopathia osteoplastica. A study of the reported cases of tracheobronchopathia osteoplastica on the one hand, and of primary localized tracheobronchial amyloidosis on the other, reveals considerable similarity and overlap between the two conditions. It is postulated that some, if not all, cases of tracheobronchopathia osteoplastica are merely the advanced ossified stage of primary localized amyloidosis of the lower respiratory tract.     

Primary amyloidoma of the axis and acute spinal cord compression: a case report

Background Localized primary amyloidosis is a disease characterized by a single tumor and localized amyloid deposit (amyloidoma) with no evidence of generalized amyloidosis. The occurrence of an amyloidoma in the spine is rare and only three cases affecting the axis have been previously reported. We describe the case of a 79-year-old woman presenting with a mass involving the odontoid process, responsible for an acute tetraparesia. Diagnosis of local primary amyloidosis was made after surgical excision.Results Despite the critical presentation, outcome was excellent after total excision of the mass. This case can be classified as a primary localized amyloidoma. The patient did not exhibit any infection, tumor or inflammatory disease, and continued investigations failed to demonstrate other amyloid deposit after one-year follow-up.Conclusions Amyloidoma must be discussed in presence of a tumor-like mass of the odontoid process and may be responsible, as in our case, for spinal cord compression.     

Primary localized amyloidosis of the nose and paranasal sinuses. A case report with immunohistochemical observations and a review of the literature.

Primary localized amyloidosis of the nose and nasopharynx is a rare disease. We present a case and review seven additional cases from the English literature. The ages of the patients ranged from 8 to 86 years; there was no sex predominance. Symptoms were nasal obstruction, epistaxis, and impaired hearing. Physical examination revealed a nasal mass or glue ears. The lesions were composed of amyloid and chronic inflammatory cells, mainly plasma cells. Ours is the first case of nasal amyloidosis in which the type of amyloid was determined immunohistochemically to be amyloid light chain (AL) lambda. The main treatment was surgical. Recurrences developed. Determination of the biochemical nature of this amyloid clarified its pathogenesis and may influence treatment. Amyloidosis should be considered in the differential diagnosis of nasal obstruction, epistaxis, and glue ears, even in the pediatric age group.     

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.     

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 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.     

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.     

A case of amyloid of the urethra and review of this rare diagnosis,its natural history and management,with reference to the literature

Primary localized genitourinary amyloid deposition is a rare disease that can be confused with cancer. Amyloid tumours of the urethra are exceptionally rare, with only 40 cases having been reported in the literature since 1909. A case is presented herein, with a full review of the presenting features, coexisting conditions and pathology and recommendations for treatment, based on the findings in previously reported cases.     

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.     

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 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.     

A case of primary diffuse tracheobronchial amyloidosis

Tracheobronchial amyloidosis (TBA) is an uncommon localized form of amyloidosis with fewer than 150 reported cases in the literature. We report a case of primary diffuse tracheobronchial amyloidosis who presented with cough, wheezing, recurrent and progressive dyspnea as well as hemoptysis. Though there is no universally accepted treatment for the localized form, this patient was successfully treated with three sessions of bronchoscopic resection and adjuvant steroids. This report will review the various types of amyloidosis of the respiratory tract and their clinical features, in addition to discussing the different available treatment modalities for TBA.     

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 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.     

A case of primitive amyloid goiter

Amyloid goiter is a rare pathology due to massive amyloid infiltration of thyroid tissue, which cause diffuse or localized enlargement of the gland. It can be totally asymptomatic or cause only non-specific symptoms (compression of adjacent structures, tracheal deviation). Thyroid disfunction (hypothyroidism or hyperthyroidism) is rare. It is important to differentiate primitive amyloid goiter from other kinds of amyloid infiltration of the thyroid, such as in systemic amyloidosis. The correct diagnosis can be difficult because of the common presence of amyloid in thyroid neoplasms, such as medullary thyroid carcinoma (MTC). The Authors present a case of primitive amyloid goiter, that needed immunohistochemical analysis for a correct diagnosis, and a review of the concerning literature.     

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 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.     

Immunoglobulin heavy chain can be amyloidogenic: morphologic characterization including immunoelectron microscopy

It is not well recognized that monoclonal immunoglobulin heavy chains or their fragments can be amyloidogenic. Amyloidosis due to heavy chains, referred to as AH amyloidosis, is rare with only three cases previously reported. An additional case of AH amyloidosis is reported. To the best of our knowledge, this is the first case of IgM heavy chain amyloidosis. A 59-year-old man presented with nephrotic syndrome. Immunoelectrophoresis detected a monoclonal IgM lambda in his serum and free lambda light chains in his urine. A renal biopsy showed amyloidosis, in which the amyloid deposits stained only for mu heavy chain by immunofluorescent and immunoelectron microscopic studies. This case suggests that monoclonal immunoglobulin heavy chains can be amyloidogenic. Furthermore, this type of amyloidosis shares the same morphologic features with other types of amyloidosis and can involve the kidney to produce nephrotic syndrome.     

Immunohistochemical Identification of Amyloid,Using an Anti‐human Serum Amyloid P Component (SAP) Antibody,is Possible in Ruminants but not in Dogs and Cats

Amyloidosis represents a heterogenous group of diseases that have in common the deposition of fibrils composed of proteins of beta‐pleated sheet structure, a structure which can be specifically identified by histochemistry using the Congo red or similar stains. Amyloid consists primarily of the amyloid fibrils but also of the amyloid P component (AP). This component, which is identical with the serum counterpart (SAP), is found in all types of human amyloid, and immunohistochemical identification of AP has been proposed as an adjunct to the universal, type‐independent diagnosis of human amyloidosis. In the present study of animal amyloidosis, we compared the amyloid‐specific Congo red stain with an immunohistochemical protocol using an anti‐human SAP antibody for the identification of amyloid in formalin fixed tissue samples. The species and types of amyloidoses investigated were: (i) seven cows, one yak (Bos grunniens), and one sheep affected with amyloidosis of presumed AA type, (ii) one dog with a pancreatic endocrine tumour producing amyloid of presumed AIAPP type, (iii) two cats with presumed AIAPP‐amyloidosis of the islets of Langerhans, one cat with presumed AA‐amyloidosis, and one cat with an amyloid‐producing odontogenic tumour. Intense immunostaining co‐localized with amyloid, identified by its congophilia and green birefringence, using a protocol without any antigen retrieval in each of the seven cows, the yak and the sheep. The method seemed more sensitive in the ruminants than the Congo red stain, but was unable to detect amyloid in the dog and the cats regardless of the application of various antigen retrieval protocols. However, specific identification of amyloid still rests on the Congo red method or similar histochemical techniques.     

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.