title: Long-term results of conformal radiotherapy for progressive airway amyloidosis.
abstract: OBJECTIVE
  To evaluate the efficacy of conformal external beam radiotherapy (RT) for local control of progressive airway amyloidosis.
  
  
  METHODS
  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.
authors.affiliation: Department of Radiation Oncology, Amyloid Treatment and Research Program, Boston University School of Medicine, Boston Medical Center, Boston, MA 02118, USA. mitruong@bu.edu
authors.forename: Minh Tam
authors.initials: MT
authors.lastname: Truong
authors.forename: Lisa A
authors.initials: LA
authors.lastname: Kachnic
authors.forename: Gregory A
authors.initials: GA
authors.lastname: Grillone
authors.forename: Harry K
authors.initials: HK
authors.lastname: Bohrs
authors.forename: Richard
authors.initials: R
authors.lastname: Lee
authors.forename: Osamu
authors.initials: O
authors.lastname: Sakai
authors.forename: John L
authors.initials: JL
authors.lastname: Berk
mesh_terms: D000328:Adult; D000368:Aged; D000686:Amyloidosis / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000532:radiotherapy*; D001982:Bronchial Diseases / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000532:radiotherapy*; D019583:Dose Fractionation, Radiation; D004941:Esophagitis / Q000209:etiology; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D007818:Laryngeal Diseases / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000532:radiotherapy*; D008297:Male; D008875:Middle Aged; D020266:Radiotherapy, Conformal / Q000009:adverse effects / Q000379:methods*; D019233:Retreatment / Q000379:methods; D012189:Retrospective Studies; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D014133:Tracheal Diseases / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000532:radiotherapy*