Search

Purpose: Airway amyloidosis is an uncommon condition and may have variable clinical course. There is no proven systemic therapy, and the management mainly relies on local invasive treatments. Several reports have found external beam radiotherapy to produce durable responses. The optimal dose and fractionation has not been determined. Endobronchial brachytherapy (EBB) has never been reported to affect this disease., Methods: The study includes a retrospective chart review of all patients with airway amyloidosis treated with EBB at the Davidoff Cancer Center. Data include symptoms, radiotherapy method and dose, treatment outcomes, and toxicities., Results: Three patients were included. Patients were either symptomatic with extensive airway involvement or have undergone multiple local procedures with rapid recurrence of the amyloid deposits. Two patients had upper and 1 patient had lower airway involvement. Two were treated with external beam radiotherapy and EBB and 1 was treated with EBB only. All patients achieved rapid improvement of symptoms with visible improvement in followup bronchoscopies. EBB doses ranged from 7.5 to 10 Gy. Of the seven lesions treated with EBB, only one lesion recurred 6 months from treatment. No major toxicities were reported., Conclusions: EBB is effective and safe for the management of airway amyloidosis and can offer long-term control. This is the first report of EBB for this indication. EBB should be further explored as means of minimizing the irradiated lung volume., (Copyright © 2018 American Brachytherapy Society. Published by Elsevier Inc. All rights reserved.)

The aim of the prospective study was to explore the safety and effectiveness of external beam radiation therapy (EBRT) in three patients with biopsy proven primary pulmonary amyloidosis, including two tracheobronchial amyloidosis patients and one primary parenchymal amyloidosis patient. All three patients were treated to 24 Gy in 12 fractions utilizing CT simulation and 3-D planning. All three patients had significant improvement in clinical symptoms, radiological imaging as well as pulmonary function tests. The improvement in the clinical symptoms was evident in 2 days. Toxicities related to EBRT were not observed during the follow-up range from 42 to 54 months. EBRT to 24 Gy was safe and effective in the three patients with primary pulmonary amyloidosis, and resulted in rapid relief of pulmonary symptoms., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

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.