Tracheobronchial Amyloidosis: Pulmonary

Tracheobronchial Amyloidosis: PulmonaryManagement has primarily consisted of debulking procedures and laser ablation. Unfortunately, such methods may be ineffective and repeated procedures may be required. Capizzi et al reported 17 patients with TBA who were treated with laser or forceps resection, systemic therapy, radiation, or observation. Of 13 patients with follow-up data, 6 patients were alive with recurrence, and 5 patients had died (recurrent respiratory compromise secondary to amyloid [n = 2] and multiple myeloma [n = 1]). Two patients remained alive with stable disease. Hui et al reported on 14 patients with tracheobronchial amyloidosis. Of the seven patients with an average follow-up of 4.8 years, three patients died of recurrent disease. Similarly, of the 10 patients recently reported by O’Regan et al at Boston University, 3 patients died of respiratory failure after numerous excisions, 2 patients have progressive disease after 11 years and 6 years of follow-up, respectively, and 1 patient requires mechanical ventilation. A review of the literature by Rubinow et al revealed that 11 of 39 patients (28%) died of respiratory causes.

Radiation therapy has a limited and variable history in the treatment of localized amyloid lesions. Pecora et al reported on the successful use of superficial radiation therapy to treat amyloidosis of the eyelid and conjunctiva. The earliest reported pulmonary cases included two patients treated for presumed primary bronchogenic carci-nomas. Shinoi et al treated a presumed primary bronchogenic carcinoma to a dose of 60 Gy using Co.
The lesion decreased in size and was found to be amyloid at resection 3 months after radiotherapy. Additional use of external beam radiation therapy is briefly mentioned in reports by Rajan et al and Thompson and Citron, but no details of technique, dose, or response were given.

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