Tracheobronchial amyloidosis: A case report and review of literature

Abstract

Tracheobronchial amyloidosis, manifested by amyloid deposits limited specifically to tracheal and bronchial tissue, is a rare manifestation with only a few hundred published cases. Patients classically present with symptoms related to fixed upper airway obstruction caused by tracheal stenosis. Clinical symptoms are non-specific and include hoarseness, dyspnea, cough, stridor, hemoptysis, and dysphagia, which are similar to those caused by more common airway disorders, often leading to incorrect, missed, and delayed diagnosis. The wide-spread use of computerized tomography (CT) imaging has the potential of dramatically advancing the early diagnosis of tracheobronchial amyloidosis. We present a case of a patient with chronic and progressive hoarseness, diagnosed with tracheobronchial amyloidosis, with a focus on unusually clear and precise CT soft tissue neck imaging. CT imaging demonstrated nodular circumferential raised mass-like thickening involving the long-segment posterior wall of the distal trachea. The wall thickening also extended into the proximal left main stem bronchi, but spared the distal bronchial tree. This resulted in moderate (approximately 50%) narrowing of the tracheal lumen, which explained the patient's hoarseness. Routine CT imaging of patients with chronic and progressive respiratory symptoms, including cough, hoarseness, and dyspnea, is recommended. Tracheobronchial amyloidosis is an uncommon disease, but it may become more commonly recognized with broader use of more effective CT imaging protocols.

A rare case of isolated laryngotracheal amyloidosis with airway narrowing and vocal fold involvement

“…When tracheal involvement is seen, the posterior membrane is not spared ( Fig. 4 ), which is in contradistinction to cartilage diseases such as polychondritis or tracheobronchopathia osteochondroplastica [9] . Direct laryngoscopic or bronchoscopic evaluation is useful for assessment of the vocal cords and glottis and to obtain tissue for biopsy.…”

A case of tracheobronchial amyloidosis presenting with acute myeloid leukemia

“…Tracheopathia osteoplastica, calcification, and ossification of subcutaneous nodules along the tracheobronchial tree can be seen in diffuse TBA 7,8 . Histopathological examination of bronchial biopsies demonstrating amyloid accumulation and Congo Red positivity leads to a definitive diagnosis 9 .…”

Primary nodular chest amyloidoma: A case report and review of literature

“…Diagnosis of localized pulmonary amyloidoma is extremely rare, in part due to the unusual nature of the condition, but also because the patients are often asymptomatic and the mass is very slow-growing. While respiratory symptoms, such as cough, dyspnea, and hemoptysis are seen in certain forms of localized pulmonary amyloidosis, such as tracheobronchial, which facilitates detection [3] , most patients with primary nodular pulmonary amyloidosis are unaware of the growth of the mass in their lung. As a result, the mass can become extremely large and destructive before it comes to medical attention, and the patients are typically older than in other forms of amyloidosis [2] .…”

“…Amyloidosis in the respiratory system involves predominantly amyloid light chain (AL) deposits and can manifest in three forms: tracheobronchial amyloidosis, diffuse alveolar-septal amyloidosis, and nodular parenchymal pulmonary amyloidoma [2] . Tracheobronchial amyloidosis is the most common form of localized primary pulmonary amyloidosis [3] . Localized nodular pulmonary amyloidoma, in which a solitary mass of amyloid is deposited in and around the lungs with no evidence of systemic amyloidosis, is extremely rare, most often asymptomatic, and may resemble primary bronchogenic carcinoma [1 , 2 , 4 , 5] .…”