International Journal of Radiation Oncology*Biology*Physics
Clinical InvestigationLong-Term Results of Conformal Radiotherapy for Progressive Airway Amyloidosis
Introduction
Amyloidoses are characterized by the extracellular deposition of insoluble fibrillar proteins in tissues and organs. Amyloid deposition in the laryngeal and tracheobronchial airways is rare and constitutes approximately 1% of benign tumors in these areas. Small populations of clonal plasma cells located outside the airway are thought to be the source of local light chain immunoglobulin amyloid formation. Laryngeal and tracheobronchial amyloid lesions can progress, leading to airway obstruction. Surgical debulking can relieve the airway obstruction caused by amyloid deposits but does not prevent recurrence (1). Low-dose radiation induces plasma cell apoptosis in other plasma cell dyscrasias and lymphoproliferative disorders, such as multiple myeloma and lymphoma 2, 3. We, therefore, hypothesized that radiotherapy (RT) could eliminate the amyloidogenic plasma cells, thereby arresting amyloid production and preventing disease progression or lesion recurrence.
Patients with tracheobronchial amyloidosis constitute approximately 11 of 1,000 referrals to the Amyloid Treatment and Research Program at Boston University School of Medicine, Boston Medical Center (1). We report the Boston Medical Center experience using low-dose external beam, three-dimensional conformal RT as localized treatment of progressive obstructive airway amyloidosis in our initial cohort of patients.
Section snippets
Study cohort
The institutional review board at Boston Medical Center approved the study as a retrospective review with a waiver of informed consent. The study followed the National Institutes of Health Insurance Portability and Accountability Act guidelines. Between 2000 and 2006, 10 consecutive patients with biopsy-proven progressive airway amyloidosis received RT. These patients were initially evaluated at the Amyloid Airway Clinic at Boston Medical Center. The histologic diagnosis of amyloidosis was
Patient and treatment characteristics
The median age at diagnosis was 39.5 years (range, 31.8–71.5). The median age at the start of RT was 40.6 years (range, 32.4–72.6). The duration of symptoms before diagnosis was 1.34 years (range, 0.34–9.29). The disease sites included 3 patients with laryngeal amyloid and 7 with tracheobronchial amyloid. Of the 10 patients, 2 were men and 8 were women. The pretreatment symptoms included bronchitis in 40%, dyspnea in 90%, cough in 40%, hemoptysis in 30%, and hoarseness in 30%. Three patients
Discussion
The present study has demonstrated that RT arrests the progression of laryngeal and tracheobronchial amyloidosis, permitting improvement in spirometry and performance status. Low-dose RT (20 Gy) achieved local disease control in 8 of 10 cases. Repeat RT alone or in combination with surgical management prevented amyloid progression in the remaining cases. The toxicities were limited to mild esophagitis in cases receiving full airway RT and small declines in DLCO, suggesting subclinical
Conclusions
Conformal RT using 20 Gy in conventional fractionation was effective in arresting progressive airway amyloidosis. The patients who responded to RT improved the functional capacity and airflow with no documented late morbidity.
Additional studies are needed to determine whether a radiation dose–response relationship for controlling airway amyloidosis exists and whether dose reductions or improvements in RT delivery techniques may reduce the potential toxicity.
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Conflict of interest: none.