Case Of Localized Amyloidosis Research Articles

Head and neck amyloidosis – report of two cases

Amyloidosis in the head and neck area is a rare, benign condition that affects mainly the larynx. Two types of amyloidosis are described in the literature, i.e., systemic and localized, with the latter being more common in the head and neck area. We report two cases of localized amyloidosis in the region of the arytenoid cartilage and palatoglossal arch. Both patients were women and had a sensation of a foreign body on swallowing. They were treated successfully surgically, and the lesions did not recur.

Huge Localized Amyloidosis of the Sinonasal Cavity: A Rare Case Report

Background: Amyloidosis is the general term for deposition of fibrous abnormal protein aggregates (amyloid) in organs or tissues that leads to dysfunction of the affected organs. Treatment and follow-up of localized amyloidosis are particularly difficult because its pathogenesis is still unclear. Localized amyloidosis of the head and neck is rare and that of the nose is rarer still. Here, we report a case of localized amyloidosis of the nasal cavity, which affected an area larger than those in previous reports. Case presentation: A 60-year-old woman presented with the chief complaints of headache, proptosis and nasal congestion. There was a lack of response to chemotherapy and endoscopic sinus surgery was performed. As of 18 months after surgery, subjective symptoms have been alleviated and are consistent with the objective findings. There have been no signs of recurrence. Conclusions: Cases of localized amyloidosis of the nasal cavity are rare, and standard treatment has not yet been established. In this case, proactive surgical intervention was successful for this condition.

Localised amyloidosis of the glans penis presenting as a painless lump with progression after 10 years

Primary amyloidosis of the genitourinary tract is uncommon, and isolated invasion of the glans penis is exceptionally rare Degos et al. (Bull Soc Fr Dermatol Syphiligr 68:159, 1961). We report a case of localised amyloidosis of the glans penis in a 40-year-old presenting as an asymptomatic penile mass which changed after 10 years prompting treatment. We believe this to be the longest interval recorded between clinical occurrence and histological diagnosis of primary penile amyloidosis.

Primary localized amyloidosis of the ureter presenting as a ureteral stone

Primary localized amyloidosis of the urinary tract is a rare disease, usually mimicking a malignancy or inflammation in its clinical presentation and in diagnostic imaging studies. We present a case of localized amyloidosis arising in the left upper ureter in a 77-year-old man. The patient visited the hospital complaining of painless gross hematuria and dysuria for 1 month. Laboratory examination revealed hematuria only. Urine cytology was unremarkable. Abdominal computed tomography revealed an obstructing mass with calcification in the ureter. The clinical impression was of a ureteral stone or malignancy. A nephroureterectomy was performed. The ureter showed segmental fibrotic thickening with luminal obstruction. Microscopic examination revealed deposition of pale-eosinophilic amorphous material with calcification and ossification. Congo-red staining showed amyloid deposits with positive polarizing microscopic findings. Although its incidence is rare, localized amyloidosis should be considered in the differential diagnosis of ureteral tumorous lesions to avoid unnecessary surgery.

Localized Oropharyngeal Amyloidosis

An otherwise healthy 29-year-old woman presented with a complaint of odynophagia of several months' duration. On examination, she was found to have a broadly based, yellow, exophytic lesion on the right lateral pharyngeal wall. Findings on biopsy were consistent with amyloidosis. The patient underwent tonsillectomy with resection of the oropharyngeal lesion. Final pathology established a diagnosis of nodular non-AL-type amyloidosis. Localized amyloidosis of the oropharynx is exceptionally rare. Additionally, most cases of localized amyloidosis are of the AL type, not the AA (i.e., non-AL) type seen in this patient.

Localized amyloidosis of the urinary bladder, clinically masquerading as bladder cancer

Amyloidosis is characterized by extracellular deposition of a proteinaceous, hyaline material. Localized deposition of amyloid in individual organs is uncommon. It can occur in the absence of systemic involvement. Herein, we describe two cases of localized amyloidosis of the urinary bladder, which clinically, radiologically, and on cystoscopy masqueraded as bladder cancer. A diagnosis of amyloidosis in both these cases was ascertained on biopsy, supplemented with special stains.

Localized Amyloidosis Of The Upper Respiratory System: Our Experience Of Three Cases And Review Of Article

Objective: To discuss the presentation, evaluation and the treatment options of localized amyloidosis affecting the upper respiratory system. Patients and Method: We conducted a retrospective study with analysis of amyloidosis by histopathologic investigations. We present three cases of localized amyloidosis; nasopharyngeal, oropharyngeal and laryngeal, with the review of literature. Results: Localized amyloidosis of the upper respiratory tract is a rare condition. The cases presented were diagnosed by histopathologic investigations. All of them were found to be free of systemic disease. They treated by surgical excision. The follow-up period ranged from two to five years. They are still being observed periodically. Conclusion: Histopathologic investigation is the gold standard for diagnosis. Simple surgical excision is the treatment for localized amyloidosis. Long term monitoring for local recurrences or subsequent development of systemic disease is mandatory. The work was done in Bakirkoy Dr. Sadi Konuk Education and Research Hospital.

Amyloidosis: Uncommon cause of dysphonia

Objectives: Case report of 3 cases of laryngeal amyloidosis Methods: Three cases of localized amyloidosis without systemic disease were identified at Centro Univerisitario de Otorrinolaringologia y Cirugia de Cabeza y Cuello at the Univerisity Hospital in Monterrey Mexico. In each patient the major site of involvement was glottic with small focus in the false vocal folds. Medical workup including full blood count, CSR, urea, electrolytes, ureanalysis, liver function test, chest x-ray, were performed. Physical examination ruled out systemic disease. Congo red staining confirmed the diagnosis in all surgical specimens. Results: Localized laryngeal amyloidosis was identified in 3 patients (2 males and 1 female) with ages ranging from 33 to 67 years. The presenting symptoms including hoarsness, throat soreness, voice breaks, and vocal fatigue. The duration of the symptoms before diagnosis varied from 3 to 12 years. Assesstment of the extent of laryngeal spread of disease included CT scan, complete voice analysis, and videolaryngostroboscopy in all patients. Material for biopsy was obtained by direct laryngoscopy, apple-green birefringence was seen with congo red stain establishing the histological diagnosis. The 3 patients underwent phonomicrosurgery for removal of the amyloid implantation using cold knife excision. Conclusion: Laryngeal amyloidosis is a rare disease with an 8 in 1,000,000 presentation rate. It is more common in males with 3:1 prevalence in the fifth decade. Once the diagnosis is confirmed by congo red staining, it is important to rule out systemic amyloid involvement. It is not necessary to do invasive approach on localized disease. Cold knife excision is still a good method to remove the deposition.

Lymphatic amyloidosis, a previously unrecognized form of amyloid deposition in generalized amyloidosis

Although amyloid deposition in relation to blood vessels is a well-recognized feature of generalized amyloidosis, lymphatic vessel amyloidosis is not mentioned in the literature. Systematic investigation of tissue removed at autopsy from patients with generalized amyloidosis and biopsy specimens from cases of localized amyloidosis and familial Mediterranean fever showed that amyloid deposition around lymphatics is by no means uncommon. The material investigated was mainly large and small bowel, lung, heart and kidney. Amyloid was identified by green birefringence with the Congo red stain on cross-polarization and lymphatics by their lack of immunostaining for CD34. Involvement of lymphatics was noted in 20 of the 42 organs from which specimens were examined, and was always accompanied by involvement of blood vessels and/or the interstitium. In the intestine, lymphatic amyloidosis was found mainly in the submucosa and subserosa, and was also demonstrated by electronmicroscopy in one case. Although lymphatic amyloidosis was equally common in the heart, lung and kidney, it was usually less prominent here than in the intestine. No lymphatic involvement was seen in localized amyloidosis. As the lymphatics play a central role in the resorption of interstitial proteins, they are probably also involved in the resorption of amyloid proteins. Amyloid deposition in the vicinity of lymphatics is probably the result of decompensation of this process.

Tracheobronchial AL amyloidosis: Histologic, immunohistochemical, ultrastructural, and immunoelectron microscopic observations

We have studied three cases of localized amyloidosis in the lower respiratory tract. Amyloid was nodularly or diffusely deposited in the lamina propria of the tracheobronchial mucosa. Its nature was confirmed by Congo red staining with green birefringence on polarized microscopy. “Tracheobronchopathia osteoplastica” also was demonstrated. Plasma cells and lymphocytes were scant in the amyloid mass. Few fibroblasts and even fewer macrophages were seen. The number of plasma cells was not increased in the bone marrow in any of our cases. Amyloid fibrils were demonstrated by electron microscopic examination. The amyloid P component was detected by immunohistochemical methods. The precursor protein of amyloidosis was shown to be amyloid L protein by the postembedding protein-A gold technique with anti-light chain antisera. The role of the plasma cells in amyloid formation, however, could not be ascertained. Based on these observations, amyloid fibril formation in tracheobronchial amyloidosis appears to be related to light chains secreted by local plasma cells, combined with amyloid P, calcium, and other factors

Amyloses localisées de découverte forfuite: analyse de 12 observations

Authors report ten cases of localized amyloidosis exposed by surgery. Three patients have developped later a systemic amyloidosis. The others patients have a local disease (2 larynx, 1 heart valves, 1 urinary bladder, 1 sciatic nerve, 1 eyelid, 1 Fallopian tube) with an excellent pronostic.

Patterns of amyloidosis and their association with plasma-cell dyscrasia, monoclonal immunoglobulins and Bence-Jones proteins.

The frequency of monoclonal immunoglobulins and Bence-Jones proteins was studied in 100 cases of amyloidosis classified according to the patterns of distribution of amyloid. Monoclonal immunoglobulins, Bence-Jones proteins (or both) were documented in all 50 Pattern I ("primary-type") cases, nine of 17 Pattern II ("secondary-type") cases, 26 of 30 Mixed Pattern I and II cases and in all three cases of localized amyloidosis. Forty-six per cent of the patients with amyloidosis (all patterns) had Bence-Jones protein only, without a monoclonal immunoglobulin, as compared with 21 per cent of cases of myeloma without associated amyloidosis. There was a higher proportion of λ-type Bence-Jones proteins in the amyloidosis group, and amyloid-related proteins were relatively more anionic than nonamyloid-related proteins. It is postulated that amyloid-related monoclonal immunoglobulins and Bence-Jones proteins may be autoantibodies or fragments of autoantibodies directed against normal tissue constituents. (N Engl J Med 290:473–477, 1974)