Laser in treatment of laryngeal amyloidosis: A case report

Amyloidosis is the extracellular deposition of the fibrinous protein amyloid in one or more body sites. Amyloidosis may broadly be classified as either primary or secondary. Primary amyloidosis is idiopathic (56%), whereas the secondary form is associated with a chronic inflammatory or infectious process (5%), Amyloidosis is also related to multiple myeloma (26%). senescence (5%) and where tumor like deposits occur in isolated organs without systemic involvement (8%). Laryngeal amyloidosis is a rare discase. Surgery has been the mainstay of treatment either endoscopically or by an external neck approach. One case of laryngeal amyloidosis, treated with endolaryngeal microsurgery and carbon dioxide laser is presented with a follow up of 8 years.     

Localized amyloidosis of upper respiratory tract

Amyloidosis results from the deposition of amyloid proteins in organ and tissues. It can be localized or systemic. Amyloidosis may be classified as either primary or secondary. Primary is idiopathic, whereas the secondary form is associated with chronic inflammatory or infectious process. Amyloidosis is also related to myeloma multiplex (plasmacytoma) or located as tumor like deposits in isolated organ without systemic involvement. Localized amyloidosis in the head and neck is rare and can have various manifestations. A case of surgically treated localized amyloidosis of the pharynx is presented. The study is focused on case history, results of laryngeal, general and pathological examination of the patient. Current options on the structure of amyloid, making diagnosis, treatment and prognosis in patients with amyloidosis are presented.     

Nasopharyngeal amyloidosis: a case report

Nasopharygeal amyloidosis is a rare entity described in the otolaryngology literature. It is usually isolated and does not represent a manifestation of systemic disease. Herein we discuss the first reported case of incidentally discovered nasopharyngeal amyloidosis leading to the diagnosis of systemic disease. A patient presented to the otolaryngology clinic with complaints of otalgia and eustachian tube dysfunction. He was noted to have a unilateral middle ear effusion and subsequent nasolaryngoscopy demonstrated a right nasopharyngeal mass at the eustachian tube orifice--this was biopsied and shown to be nasopharyngeal amyloidosis. Amyloidis is a condition which entails the abnormal deposition of proteins in tissue (local) and organs (systemic) throughout the body secondary to an alteration in the secondary structure of the protein into an insoluble form, termed amyloid. Amyloidosis in the head and neck most often presents in the larynx (60%), followed by the trachea (9%), orbit (4%), and nasopharynx (3%). The vast majority of these lesions represent isolated amyloidosis, which is not associated with a shortened lifespan or the sequelae associated with systemic disease. Treatment of local disease is typically excision followed by routine surveillance for recurrence. Treatment of systemic disease is complex, and should be managed by a Hematologist/Oncologist. Amyloidosis can be associated with malignancy, thus proper workup is important even when isolated lesions are discovered.     

Localized amyloidosis of the tongue: a review

Amyloidosis is a disease entity defined by the presence of extracellular deposits of proteinacious material. These deposits have a characteristic apple-green birefringence with polarized light after staining with Congo red. Amyloid involvement of the tongue is almost universally secondary to systemic disease. The mean survival of patients with a systemic form of amyloidosis is between 5 to 15 months, whereas those with the localized form have an excellent prognosis. This article presents a case of the more unusual localized form. A thorough evaluation, including abdominal fat or rectal biopsy, is essential in every patient to identify any systemic involvement. The absence of systemic amyloidosis offers a much more favorable prognosis and may be treated with simple surgical excision.     

Primary salivary gland amyloidosis causing sicca syndrome.

Sicca syndrome (SS), consisting of xerostomia and xerophthalmia, may be caused by various disease processes. We present a unique case of SS secondary to primary amyloidosis. Amyloidosis is a rare but definite cause of SS and should be included in the differential diagnosis of any patient who presents with sicca symptoms. A literature review comparing amyloidotic patients with SS and patients with amyloidosis only demonstrates that both of these groups of patients present similarly with regard to symptoms. However, the majority of patients with SS present with sicca symptoms initially in addition to symptoms of amyloidosis. These SS patients also present with proteinuria and negative serology test results. Therefore, patients presenting with sicca symptoms, proteinuria, and negative serologic findings should be suspect for amyloidosis. The importance of distinguishing the diagnosis of Sj?gren's syndrome from SS in these patients cannot be overemphasized. There is a significantly higher incidence of developing a lymphoma in Sj?gren's syndrome patients. This has important implications for the head and neck surgeon treating these patients.     

Amyloidosis of the larynx

The paper presents three cases of localised amyloidosis of the larynx. The study was focused on case histories, findings of larynx examination and method of treatment was discussed. Amyloidosis is a disease characterised by gathering of amyloid deposits in extracellular space. The amyloid fibres consist of different kinds of proteins which have the ability of forming laminae. This disease may be secondary to numerous general processes in which amyloid proteins forms. Etiopathogenesis of this disease has not been explained yet. In the following paper the latest theories and classification of amyloidosis are presented.     

Nasopharyngeal amyloidosis

PURPOSE: To discuss the presentation of localized amyloidosis affecting the nasopharynx and discuss the management options. Amyloidosis in the head and neck is a rare and benign condition that usually takes the form of localized amyloidosis. Because systemic amyloidosis markedly shortens life expectancy owing to its involvement with vital organs, rectal biopsy or fat aspiration of the anterior abdominal wall must be carried out to exclude systemic involvement. Localized amyloidosis in the head and neck can involve the orbit, sinuses, nasopharynx, oral cavity, salivary glands, and larynx. METHODS: We present the case of a patient with conductive hearing loss and serous otitis media with effusion secondary to nasopharyngeal amyloidosis, as well as present a review of the literature. RESULTS: Only a few cases of nasopharyngeal amyloidosis have thus far been reported. Patients with this disease can also present with recurrent epistaxis, postnasal drip, nasal obstruction, and eustachian tube dysfunction. Localized amyloidosis of the nasopharynx, which is slow growing, has proved difficult to treat because it can persist or recur despite surgical treatment. Furthermore, bleeding may be a major complication in treating patients with nasopharyngeal amyloidosis by transpalatal excision because the amyloid deposits cause vascular wall fragility. Finally, there is no evidence that surgical treatment of nasopharyngeal amyloidosis can prolong survival or that localized amyloidosis can progress to systemic amyloidosis. For these reasons, we elected to treat our patient with a tympanostomy tube and observation. CONCLUSION: In the absence of systemic disease, localized amyloidosis of the nasopharynx may be treated conservatively.     

Amyloidosis of the larynx

Amyloidosis of the larynx is a rare disease, accounting for less than 1% of all benign laryngeal 'tumours'. Three cases of this type of lesion are reported--one of the vocal cord, one of the false vocal cord and one of the subglottis and trachea. In 2 of the patients the amyloidosis was localized, while the third was later found also to have an epipharyngeal solitary plasmacytoma with amyloid deposits and in addition amyloidosis of the nasal cavity. However, the amyloidosis in this patient may still be regarded as being localized, as the clinical examination and laboratory tests afforded no evidence of generalized disease. Amyloidosis of the larynx may be manifested as a localized tumour or as a diffuse infiltration. The symptoms and signs will, of course, depend on the site of the amyloid deposit. When the vocal cords are involved hoarseness may result, and this was the most prominent sign in the present cases. The treatment of laryngeal amyloidosis is primarily by endoscopic excision. Amyloid substance has specific staining properties. The Congo red reaction with a green birefringence in polarized light and Phorwhite BBU using fluorescence microscopy are regarded as the most reliable staining reactions. Electron microscopy has revealed a typical fibrillar structure of amyloid.     

Macroglossia secondary to systemic amyloidosis: case report and literature review

Amyloidosis is characterized by an abnormal extracellular deposition of amyloid in different tissues and organs, where it usually causes some type of dysfunction. Its cause is unknown. The two main forms of amyloidosis are systemic and localized; the latter is rare. No satisfactory treatment for systemic amyloidosis has been discovered, and mean survival is poor, ranging from 5 to 15 months depending on the presence or absence of multiple myeloma. We report a case of primary systemic amyloidosis in a 71-year-old man. The diagnosis of amyloidosis was established by tongue biopsy, and its systemic nature was identified by analysis of aspirated abdominal fat. At the 1-year follow-up, the patient's clinical condition had not changed, and he was thereafter lost to follow-up.     

Management of laryngeal amyloidosis

Amyloidosis of the upper aerodigestive tract is rare. However, the larynx is the commonest site within the upper airways to be affected. Three cases of uncomplicated primary amyloidosis of the larynx and one case of laryngeal amyloidosis with more generalized dissemination throughout the head and neck are presented. In this article the different presentations of laryngeal amyloidosis and principles of management of this condition at that site are highlighted.     

Amiloidosis del canal auditivo externo

Amyloidosis involving the external ear is extremely rare. We present the case of a 76-year-old man who referred unilateral otorrhea and hypoacusis of six months’ duration. The external auditory canal (EAC) was narrowed by a subcutaneous nodule. After its extirpation, unilateral localised amyloidosis of the EAC was diagnosed. In these rare cases, it is crucial to rule out systemic amyloidosis.     

Localized amyloidosis masquerading as nasopharyngeal tumor: a review

Amyloidosis comprises a diverse collection of disease characterized by the presence of amorphous extracellular eosinophilic deposits of unique protein fibrils that gives apple green birefringence under polarized light after staining with Congo red. Head and neck region is the commonest site for localized form of amyloidosis. We report a case of a 43-year-old man with localized amyloidosis of nasopharynx with oropharyngeal extension and its management, along with a review of relevant literatures.     

Amyloidosis of the upper aerodigestive tract

OBJECTIVES/HYPOTHESIS: To delineate the clinical and pathologic characteristics of upper aerodigestive tract amyloidosis with particular attention to laryngeal amyloidosis. STUDY DESIGN: Retrospective chart review of patients with amyloidosis of Thomas Jefferson University and its affiliated hospitals. MATERIAL AND METHODS: The charts of 16 patients with upper aerodigestive tract amyloidosis identified from the databases of the Thomas Jefferson University pathology department were reviewed and included in the study. RESULTS: Sixteen patients (9 male and 7 female, with an average age of 49.8 years) with upper aerodigestive tract amyloidosis were identified. The most common site of amyloid involvement was the larynx. Consequently, patients most commonly presented with hoarseness (14 of 16). All patients underwent surgical removal of the amyloid deposits. Fourteen patients had primary localized amyloidosis. Two experienced systemic involvement. Seven of the 16 patients developed recurrences requiring further treatment. CONCLUSIONS: Amyloidosis of the upper aerodigestive tract generally behaves as a benign, localized condition treatable by surgical resection. Regular follow-up with laryngoscopy is indicated for early diagnosis of recurrence, and multiple surgical procedures may be required to control symptoms.     

Amyloidosis of the external auditory canal and middle ear: unusual ear tumor

Amyloidosis of the ear is rare. We describe the case of a 41-year-old man who had localized amyloidosis that involved the external auditory canal and middle ear. To the best of our knowledge, only 4 other cases of amyloidosis involving the external auditory canal have been previously reported; in none of these cases was the middle ear involved. We also discuss the clinical importance of this condition and its treatment.     

Amyloidosis of Waldeyer's ring and larynx

Amyloidosis of the upper aerodigestive tract is relatively rare. A case of localized amyloidosis involving all components of Waldeyer's ring with added laryngeal involvement is described. This has not been previously reported. A literature review of this conditions is presented.     

Amyloidosis in the ORL field

ObjectivesTo review the clinical and pathological characteristics of upper aerodigestive tract amyloidosis with particular attention to laryngeal amyloidosis. Amyloidosis of the upper aerodigestive tract is relatively rare. The larynx is the most common site of involvement in head and neck isolated amyloidosis and the supraglottic region represents the major site of involvementMaterial and MethodsRetrospective review of 6 patients diagnosed with upper aerodigestive tract amyloidosis. Hoarseness and airway compromise were the main presenting symptomsResultsLaryngeal CO2 laser microsurgery was perfomed and then we refered the patients to the Medical Deparment seeking for systemic involvement and ENT Clinic follow upConclusionsIn our experience, laryngeal CO2 laser microsurgery is a succesfull way to treat isolated laryngeal amyloidosis with clinical improvement and low recurrence rates     

Endoscopic management of laryngo-tracheobronchial amyloidosis: a series of 32 patients

Between June 1983 and December 2002, 32 patients were treated for primary localized laryngo-tracheobronchial amyloidosis (LTBA) at our institution. For enrollment in this retrospective study, at least one positive biopsy with Congo red stain and a diagnostic test battery excluding systemic or secondary amyloidosis were mandatory. The most common presenting symptom was dyspnea, which was observed in 75% of cases. Endoscopic appearance was described as submucosal plaques and nodules with a cobblestone appearance in 44% of patients, tumor-like in 28% and circumferential wall thickening in 28% of cases. Tracheobronchopatia osteochondroplastica was associated with LTBA in 22% of patients, both at the first treatment (four cases) or during follow-up examinations (three). Synchronous or metachronous disease in the larynx and tracheobronchial tree was observed in 47% of cases, while only one patient showed lung involvement with extensive amyloidosis of the trachea and bronchi. All but two patients were endoscopically treated. One of these was affected by a tracheal amyloidoma cured by endoscopic debulking and further tracheal resection-anastomosis. The other patient presented massive laryngo-pharyngeal involvement and was therefore treated by total laryngectomy. A total of 58 endoscopic procedures (range: 1-7; mean: two per patient) were carried out, 11 with CO2 laser (for supraglottic, glottic and selected subglottic lesions) and 47 with Nd:YAG laser (for the most part of subglottic lesions and amyloid deposits located in trachea and bronchi). Four patients required a tracheotomy and three necessitated a T-tube stent during management, which was subsequently removed in all cases. One patient was lost to follow-up, nine were endoscopically controlled elsewhere (Group A) and 22 were followed at our institution (Group B). In Group A, the patient with tracheal amyloidoma is asymptomatic and free of additional LTBA localizations 8 years after surgery. The remaining eight are asymptomatic, but with persistent endoscopic signs of amyloidosis. In Group B, five patients died: one from heart failure and another one from pneumonia 3 and 4 days after surgery, respectively. In one case death was from unrelated causes, and in the two remaining patients it was from respiratory failure due to uncontrolled bilateral bronchial amyloidosis in the subsequent 5 to 6 years. One patient is completely cured 5 years after surgery, and 16 are asymptomatic with persistence of LTBA.     

Multifocal localized amyloidosis in a pediatric patient

Amyloidosis of the upper airway is rare in the pediatric age group. We present a primary amyloidosis in the nasopharynx and nasal cavity in a 14-year-old girl. The symptoms were nasal obstruction and mucoid discharge. The diagnostic was revealed by histologycal analysis obtained from local surgical excision.     

The value of biopsy of the accessory labial salivary glands for the diagnosis of amylosis]

Diagnosis of amyloidosis depends on the demonstration of amyloid deposits in biopsies using specific stains. Recently, in addition to classical biopsies (kidney, liver, gum, skin, rectal mucosa), labial salivary gland biopsy has been recommended as safe diagnostic method. In our recruitment, it allowed the fortuitous discovery of amyloidosis in three patients suffering from rheumatoid polyarthritis or spondylarthritis. In five other patients (2 cases of familial amyloidosis, 1 dysglobulinemia, 2 primary cardiac amyloidosis), biopsy was performed for systematic search of amyloidosis. In five of these eight cases, a sicca syndrome was associated with the salivary deposits. These deposits were stained with congo red viewed in polarized light and with T thioflavine. Besides, Wright's method allowed to know the AL or AA type of amyloidosis and thus to guide the treatment. On the whole, labial salivary gland biopsy is a highly sensitive method for diagnosis of primary and secondary amyloidosis.     

Beta-2-microglobulin as the main component of primary amyloidosis located in the larynx

A case of primary amyloidosis located in the larynx in which Beta-2-microglobulin (β2m) was the main component of the tissue is presented. Indirect immunofluorescence performed with monoclonal anti-human β2m antibody (BBM1) showed a positive reaction, as did Congo red stain, and both patterns were similar. A significant increase in serum β2m was observed; however, the clinical condition associated with this elevation remains unclear. We suggest that β2m is not only the main component of secondary amyloidosis in terminal nephropathy patients undergoing hemodialysis for prolonged periods, but that it could be part of the structure of other types of amyloid—either primary or secondary.