Long-Term Results of Conformal Radiotherapy for Progressive Airway Amyloidosis

doi:10.1016/j.ijrobp.2011.07.036

International Journal of Radiation OncologyBiologyPhysics

Volume 83, Issue 2, 1 June 2012, Pages 734-739

Presented in part at the 49th Annual Meeting of the American Society for Radiation Oncology, October 28, 2007 to November 1, 2007, Los Angeles, CA.

https://doi.org/10.1016/j.ijrobp.2011.07.036 Get rights and content

Purpose

To evaluate the efficacy of conformal external beam radiotherapy (RT) for local control of progressive airway amyloidosis.

Methods and Materials

We conducted a retrospective review of patients with biopsy-proven progressive airway amyloidosis treated with conformal RT between 2000 and 2006 at Boston Medical Center. The patients were evaluated for performance status and pulmonary function, with computed tomography and endoscopy after RT compared with the pretreatment studies. Local control was defined as the lack of progression of airway wall thickening on computed tomography imaging and stable endobronchial deposits by endoscopy.

Results

A total of 10 symptomatic airway amyloidosis patients (3 laryngeal and 7 tracheobronchial) received RT to a median total dose of 20 Gy in 10 fractions within 2 weeks. At a median follow-up of 6.7 years (range, 1.5–10.3), 8 of the 10 patients had local control. The remaining 2 patients underwent repeat RT 6 and 8.4 months after initial RT, 1 for persistent bronchial obstruction and 1 for progression of subglottic amyloid disease with subsequent disease control. The Eastern Cooperative Oncology Group performance status improved at a median of 18 months after RT compared with the baseline values, from a median score of 2 to a median of 1 (p = .035). Airflow (forced expiratory volume in 1 second) measurements increased compared with the baseline values at each follow-up evaluation, reaching a 10.7% increase (p = .087) at the last testing (median duration, 64.8 months). Acute toxicity was limited to Grade 1-2 esophagitis, occurring in 40% of patients. No late toxicity was observed.

Conclusions

RT prevented progressive amyloid deposition in 8 of 10 patients, resulting in a marginally increased forced expiratory volume in 1 second, and improved functional capacity, without late morbidity.

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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Citation Excerpt :
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: Conflict of interest: none.

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Clinical InvestigationLong-Term Results of Conformal Radiotherapy for Progressive Airway Amyloidosis

Purpose

Methods and Materials

Results

Conclusions

Introduction

Section snippets

Study cohort

Patient and treatment characteristics

Discussion

Conclusions

Chest

Mayo Clin Proc

Chest

Chest

Urology

Mayo Clin Proc

Blood

Int J Radiat Oncol Biol Phys

Int J Radiat Oncol Biol Phys

Tracheobronchial amyloidosis: The Boston University experience from 1984 to 1999

Medicine (Baltimore)

Prognostic factors in solitary plasmacytoma of the bone: A multicenter Rare Cancer Network study

BMC Cancer

[Role of radiotherapy in the treatment of multiple myeloma]

Strahlenther Onkol

Clinical Investigation
Long-Term Results of Conformal Radiotherapy for Progressive Airway Amyloidosis