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Trachea

Non-neoplastic lesions

Amyloidosis

Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 4 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
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● Presents as either diffuse narrowing (circumferential thickening of wall) of airway or solitary / multiple nodules (pseudotumoral)
● Associated with laryngeal or nasal involvement

Clinical features
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● Symptoms of asthma, atelectasis, hemoptysis, obstruction
● May induce tracheomalacia in rheumatoid arthritis
● May be due to myeloma, lymphocytic interstitial pneumonia, lymphoplasmacytic lymphomas, plasma cell dyscrasias
● Does not usually evolve into systemic amyloidosis
● 15-40% die at mean 9 years after diagnosis from respiratory failure, pulmonary hemorrhage, pneumonia

Case reports
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● 49 year old man with chronic cough and left lung hilar mass (Arch Pathol Lab Med 2003;127:e420)
● 50 year old man with nasal obstruction (Nihon Kyobu Shikkan Gakkai Zasshi 1997;35:1378)
● 53 year old man with tracheobronchopathia osteochondroplastica and AA amyloidosis (Yonsei Med J 2009;50:721)
● 60 year old woman with pseudotumoral tracheobronchial amyloidosis mimicking asthma (J Med Case Rep 2012;6:40)
● 64 year old woman with history of myasthenia gravis and type 1 diabetes mellitus (AJNR Am J Neuroradiol 2007;28:1557)
● 69 year old woman with seropositive erosive RA and bronchopneumonia (Clin Rheumatol 2008;27:807)
● 70 year old man with tracheobronchial circumferential wall thickening and mediastinal fat infiltration (Clin Nucl Med 2011;36:723)
● Cases with involvement of upper third of the trachea (Vestn Otorinolaringol 2008;(1):67)
● Atypical case necessitating tracheotomy (Eye Ear Nose Throat Mon 1952;31:193)

Treatment
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● Laser therapy or bronchoscopic removal of deposits, radiation therapy, lung transplant

Gross description
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● Focal to diffuse nodular thickening of trachea and proximal bronchial walls with patchy mural calcification
● Also extensive bronchial stenosis, postobstructive pneumonia, atelectasis

Micro description
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● Extensive thickening of submucosa due to irregular nodular masses or sheets of amyloid, reduced submucosal glands, calcification or osseous metaplasia of larger airways
● Variable multinucleated, osteoclast-like giant cells and plasma cells within amyloid
● Also amyloid deposition within submucosal vessel walls

Micro images
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Figure 1: bronchial margin; 2: bronchi; 3: bronchi H&E; 4: Congo Red

   
Homogenous proteinous material with calcification under bronchus epithelium is Congo Red+


Massive amyloid deposits stain with anti-lambda light chain antibodies


69 year old woman with seropositive erosive RA

Positive stains
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● Congo Red (apple-green birefringence with polarized light)

Differential diagnosis
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● Light chain deposition disease
● Pulmonary lymphoproliferative disorders
● Pulmonary scar tissue
● Systemic amyloidosis
● Tracheobronchopathia osteochondroplastica: submucosal bony and cartilaginous tissue projects into tracheobronchial lumen, no amyloid

End of Trachea > Non-neoplastic lesions > Amyloidosis

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