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A3155 - Everything that Wheezes Is Not Asthma: Rare Case of Primary Endobronchial Amyloidoma
Author Block: A. Lal1, J. Akhtar1, Y. Chen2, Y. Goldman3; 1Department of Medicine, Saint Vincent Hospital, Worcester, MA, United States, 2Department of Pathology, Saint Vincent Hospital, Worcester, MA, United States, 3Reliant Medical Group, Worcester, MA, United States.
Introduction: Primary tracheobronchial amyloidosis is a rare disease with extracellular amyloid deposits. With the advancements in fiberoptic bronchoscopic techniques and improvement in imaging modalities more and more cases are being reported.Case: A 65 year old man was being treated for poorly controlled asthma with worsening over past 4 years. He was a former smoker with smoking history of 40 pack years, and had quit smoking 20 years ago. Radioallergosorbent test was normal and immunoglobulin E levels were within normal limits. There was no eosinophilia. Pulmonary function tests done were indicative of mild obstructive physiology with no significant change in volumes or flow after bronchodilator administration. As there was persistent wheezing and shortness of breath, CT chest was done, which revealed left upper lobe bronchial wall thickening and calcification with moderate stenosis. In view of abnormal bronchial wall thickening patient underwent flexible bronchoscopy, which revealed slight yellowish color, nodular tumor like lesion in left upper lobe. Endobronchial biopsy was done with a suspicion of malignancy, especially with history of smoking. Biopsy of the lesion showed benign bronchial mucosa and underlying stroma with pink amorphous material deposit showing orange red by congo red stain and green birefringence under polarized light classical for amyloidosis. Serum and Urine electrophoresis was normal. Discussion: Primary tracheobronchial amyloidosis (PTBA) is extremely uncommon with only around 150 cases reported in the literature. Out of all symptomatic tracheobronchial lesions, only 0.5% are amyloid lesions. Primary endobronchial amyloidosis is characterized by submucosal plaques or nodules which may be localized, diffuse or multifocal. Solitary localized endobronchial amyloid nodule or tumor mimicking an endobronchial neoplasm is even rarer, presenting in only 14% of the tracheobronchial amyloidosis cases. Tracheobronchial amyloidosis can be asymptomatic or can present with nonspecific symptoms, mimicking common respiratory conditions such as asthma, COPD, pneumonia. The diagnosis is usually delayed by 8-37 months. So, for patients with asthma, chronic cough, COPD or recurrent pneumonia who do not show anticipated response to treatment, we should do complete workup for primary tracheobronchial amyloidosis with CT Chest, bronchoscopy and biopsy if required. There is no established treatment for PTBA. For symptomatic patients with significant airway obstruction, bronchoscopic debulking, silicon stenting and laser ablation are the mainstay of treatment. External beam radiotherapy has been used in few cases of endobronchial amyloidosis successfully. Our patient declined any invasive treatment at a higher center of care and is currently managed conservatively.