Chest
Volume 125, Issue 2, February 2004, Pages 784-789
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Selected Reports
Tracheobronchial Amyloidosis: A Case Report of Successful Treatment With External Beam Radiation Therapy

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Tracheobronchial amyloidosis (TBA) refers to the deposition of localized amyloid deposits within the upper airways. Treatments have historically focused on bronchoscopic techniques including debridement, laser ablation, balloon dilation, and stent placement. Local excisions often prove temporarily effective, with multiple local recurrences and progressive compromise pulmonary function occurring frequently. We present a case of TBA managed with definitive external beam radiation therapy. Eighteen months after moderate-dose radiation, the patient demonstrated improvements in functional status, pulmonary function, bronchoscopic visualization, and CT-based luminal diameters. The literature involving the role of radiation therapy in the treatment of TBA is reviewed.

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Case Report

The patient is a 60-year-old white woman with multiple medical comorbidities, including COPD, hypertension, diabetes mellitus, coronary artery disease, congestive heart failure, and an 80-pack-a-year history of cigarette smoking. She presented in June 2001 to an outside institution with a 3-week history of increasing shortness of breath, cough productive of white sputum, and occasional hemoptysis. The patient required 4 L/min of oxygen by nasal cannula at rest to maintain adequate oxygenation.

Discussion

TBA is a relatively rare disorder characterized by submucosal amyloid deposition in the airways ranging from the larynx to the distal airways. Commonly associated symptoms include dyspnea, nonproductive cough, hemoptysis, and hoarseness. Diagnosis is frequently delayed by up to 17 months,3 and is verified by green birefringence of Congo red-stained areas when viewed with polarized light.

TBA along with nodular and diffuse alveolar septal parenchymal involvement represent the three primary

Conclusion

A case of external beam radiation therapy for amyloidosis localized to the tracheobronchial tree is presented. We report improvements in functional status, pulmonary function, bronchoscopic visualization, and CT-based luminal diameters after moderate-dose radiation therapy. External beam radiation therapy may prove more definitive than current management techniques and warrants further investigation.

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