Long-term results of conformal radiotherapy for progressive airway amyloidosis.

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.

Recombinant human thyrotropin-stimulated radioiodine therapy of large nodular goiters facilitates tracheal decompression and improves inspiration.

INTRODUCTION: The impact on tracheal anatomy and respiratory function of recombinant human (rh)TSH-stimulated (131)I therapy in patients with goiter is not clarified. METHODS: In a double-blinded design, patients (age 37-87 yr) with a large multinodular goiter (range, 99-440 ml) were randomized to placebo (n = 15) or 0.3 mg rhTSH (n = 14) 24 h before (131)I therapy. The smallest cross-sectional area of the trachea (SCAT; assessed by magnetic resonance imaging) and the pulmonary function were determined before, 1 wk, and 12 months after therapy. RESULTS: Data on goiter reduction have been reported previously. In the placebo group, no significant changes in the lung function or SCAT were found throughout the study. In the rhTSH group, a slight decrease was observed in the forced vital capacity 1 wk after therapy, whereas the mean individual change in SCAT was significantly increased by 10.5% (95% confidence interval = 0.9-20.0%). A further increase in SCAT to 117 +/- 36 mm(2) (P = 0.005 compared with 92 +/- 38 mm(2) at baseline) was seen at 12 months, corresponding to a mean of 31.4% (95% confidence interval = 16.0-46.8%). The expiratory parameters did not change significantly, whereas forced inspiratory flow at 50% of the vital capacity (FIF50%) increased from initially 3.34 +/- 1.33 liters/sec to ultimately 4.23 +/- 1.88 liters/sec (P = 0.015) in the rhTSH group, corresponding to a median increase of 24.6%. By 12 months, the relative improvements in FIF50% and in SCAT were inversely correlated to the respective baseline values (FIF50%: r = -0.47, P = 0.012; SCAT: r = -0.57, P = 0.001). CONCLUSION: On average, neither compression of the trachea nor deterioration of the pulmonary function was observed in the acute phase after rhTSH-augmented (131)I therapy. In the long term, tracheal compression is diminished, and the inspiratory capacity improved, compared with (131)I therapy alone.

Tracheobronchial amyloidosis: utilization of radiotherapy as a treatment modality.

Tracheobronchial amyloidosis (TBA) is a rare disease. No general consensus exists with regard to its optimal treatment, resulting in a variety of modalities used to manage this condition. In this article, we present a case of TBA treated with external beam radiation therapy with encouraging results. A brief literature review of this rare ailment is also included.

Tracheobronchial amyloidosis: a case report of successful treatment with external beam radiation therapy.

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.

Paratracheal extramedullary hematopoiesis.

Extramedullary hematopoiesis (EMH) is a rare finding in hematology. A 73-year-old female patient with a 1-week history of severe progressive dyspnea was examined, and computed tomography (CT) showed a paratracheal mass 3 cm in size located 1 cm below the vocals cords and causing obliteration of the tracheal airway. Cytology of a needle biopsy revealed EMH. External radiotherapy of 200-cGy fractions to a total dose of 2000 cGy was administered with 3-dimensional conformal planning to treat the progressive symptoms. The patient's clinical symptoms started to improve 2 days after radiotherapy and had completely disappeared after 7 days. CT scans showed complete response on follow-up at 1 week to 5 months after radiotherapy. Mature and immature hematopoietic cells and many adipose cells were seen in the pretreatment samples. Histologic findings in the posttreatment samples showed that these cells had completely disappeared due to the conformal radiotherapy. On the basis of clinical, radiologic, and histologic results, we suggest that conformal radiotherapy may be useful for the treatment of paratracheal localization of EMH because good tumoral irradiation was obtained in this case, with the protection of normal tissues.

External-beam radiation therapy in the treatment of diffuse tracheobronchial amyloidosis.

Tracheobronchial amyloidosis is characterized by deposits of amyloid in airway walls. No effective treatment is known. We describe a 59-year-old woman who presented with increasing symptoms of airway obstruction due to diffuse deposition of amyloid throughout her tracheobronchial tree. She was treated with external-beam radiation therapy (20 Gy) with marked improvement in her symptoms, effort tolerance, bronchoscopic appearance, and forced expiratory volume in 1 second (1.39 L to 1.97 L [42%]). This improvement was maintained during 21 months of follow-up.

Treatment of endotracheal or endobronchial obstruction by non-small cell lung cancer: lack of patients in an MRC randomized trial leaves key questions unanswered. Medical Research Council Lung Cancer Working Party.

Symptoms of endotracheal or endobronchial obstruction caused by non-small cell lung cancer (NSCLC) may be relieved with external beam radiotherapy (XRT) or endobronchial treatment. The comparative roles of these two methods need to be established. Patients with predominantly intraluminal obstruction of the trachea, a main bronchus or a lobar bronchus by unresectable NSCLC were randomized to XRT versus the clinician's choice of endobronchial treatment with brachytherapy, laser resection or cryotherapy, according to local availability and practice. Clinicians' assessments included symptoms of obstruction, WHO performance status, lung function tests and adverse effects of treatment. Patients completed a Rotterdam Symptom Checklist at all assessments and a daily diary card to record the severity of major symptoms during the first 4 weeks. To show a difference of 15% in the relief of breathlessness rates at 4 months (from 65% to 80%), 400 patients were required. In spite of our many previously successful lung cancer trials, and initial interest from clinicians in 24 UK centres, who estimated they could randomize 200 patients per year into the present trial, only 75 patients were randomized from seven centres over 3.5 years. Intake to the trial was therefore abandoned in November 1996 although an independent Data Monitoring and Ethics Committee had concluded in April 1996 that the scientific case for the trial was still strong; there were no competing trials; there were no design problems; and much had been done to promote the trial. The main reasons given by centres for the slow intake were: lack of referrals of untreated patients; patients being referred specifically for endobronchial treatment; patients having already received XRT; emergency endobronchial relief of obstruction being necessary; and XRT and endobronchial treatment being considered complementary and not as alternatives. The relative advantages and disadvantages of XRT versus endobronchial treatment remain to be determined. The lack of recruitment to this trial raises the issue of innovative techniques not being given the chance of proving their worth compared with traditional treatments.

Radiation therapy for tracheobronchial amyloidosis.

A 67-year-old man presented with localized tracheobronchial amyloidosis involving the distal trachea and the right-sided airways. The disease caused right middle lobe collapse and threatened the right upper and lower lobes. A variety of bronchoscopic methods, including Nd:YAG laser resection, dilation, and stenting, were used as temporizing methods. External beam radiation therapy, considered because of disease progression, caused a measurable local response. Radiation therapy should be considered as a treatment option for localized tracheobronchial amyloidosis causing airway obstruction.

[Nd-YAG laser treatment of tracheobronchial lesions].

From January 1987 to May 1988, 32 cases with tracheobronchial lesions were treated with Nd-YAG laser. Complete restoration of the airway was achieved in one case of leiomyoma, adenoma, carcinoid and mucous epithelioma each, 2 cases of polyp and 7 cases of granuloma. Significant restoration was seen in one case of granuloma, 2 cases of hematoma and papilloma, each, 3 cases of amyloidosis and partial restoration in one case of granuloma. The remaining 10 cases with squamous cell carcinoma and adenocarcinoma with severe airway obstruction were treated by Nd-YAG laser for palliative purpose only. Four of them revealed significant remission and 6 responded with partial remission. No severe complication was observed during or after the procedures. Nd-YAG laser treatment provides a new curative method avoiding resection of the lung in benign lesions and a palliative therapy for unresectable obstructive malignant tumors.

Laser photoradiation therapy for tracheobronchial amyloid.

Tracheobronchial amyloid is uncommon and has a high morbidity and mortality. In the past, progressive airways obstruction has been treated with bronchoscopic resection but benefit has been limited. We report the successful use of a laser to treat this condition and the responses of amyloid tissue to laser photoirradiation.

Amyloid pseudotumor treated by tracheal resection.

Primary amyloidosis is a rare disease, and even more so when it appears as a tumorlike formation in the lung or tracheobronchial tree. On rare occasions it has been treated by pulmonary resection but never before by tracheal or bronchial sleeve resection. A case of tracheal sleeve resection for tumorlike amyloidosis is reported. If the lesion is well circumscribed to a relatively short tracheal or bronchial segment, sleeve resection appears to be a satisfactory and economical solution. However, the problem of a mucosa undermined by amyloid deposits above and below the tumorlike lesion entails the risk of anastomotic stenosis. For this reason in future cases, particularly those with extensive involvement, piecemeal endoscopic removal should be considered as the method offering the best prognosis and least risk.