A 50 year old woman was admitted to the hospital for bronchoscopic evaluation. She was an active smoker with a cigarette smoking history of 30 pack years and had suffered from repeated bronchitis, shortage of breath and hoarseness as well as repeated episodes of hemoptysis. She also complained of nasal congestion and recurrent sinusitis. A pulmonary infiltrate in the right upper lobe had not resolved after antibiotic treatment.
On clinical examination, stridor was audible in the central airways. The physical examination of other organ systems was not remarkable. Laboratory investigations included normal blood count, normal erythrocyte sedimentation rate and normal biochemistry. A computer tomography scan revealed an atelectasis of the right upper lobe and diffuse nodular thickening of the submucosal tracheal and bronchial walls (Fig. 1). The cartilage showed calcifications. There were no other parenchymal abnormalities. Arterial blood gases, while breathing room air, were normal. Pulmonary function tests revealed normal FVC, FEV1, FEV1/FVC; but decreased PEF and an expiratory plateau in the flow volume loop, suggestive of intrathoracic stenosis (Fig. 2).
Flexible bronchoscopy was performed and a completely irregular surface of the tracheal and bronchial mucosa with prominent white and reddish plaques, extending the lower half of the trachea and main bronchi with reduced airway diameters and diffuse bleeding was found (Fig. 3). Biopsies showed amorphous deposits, identified as amyloid by apple-green birefringence in sections stained with congo red and seen under polarized light. Immunohistochemical staining demonstrated AL amyloid. Systemic amyloidosis was excluded by normal serum protein electrophoresis, normal urine analyses (no Bence Jones protein in the urin), normal rectal biopsy specimens, normal bone marrow aspiration/biopsy, normal echocardiography and abdominal ultrasound.
The patient underwent bronchoscopic debridement with forceps debulking for more extensive bioptic work-up and treatment of airway stenosis. The procedure was complicated by prolonged but self-limited bleeding. The case was discussed at the multidisciplinary respiratory oncology board and it was felt that external beam radiation therapy (EBRT) was an appropriate treatment option. Radiation therapy was explained in detail to the patient because of the scarcity of clinical data regarding its use for TBA. She received EBRT, 20 Gy in 10 fractions. Opposed anterio-posterior and posterior-anterior treatment fields were used to encompass the entire tracheobronchial tree. The treatment volume was determined using CT-guided simulation. Treatment was uneventful. Clinically her symptoms improved and the pulmonary function test stabilized during 12 month follow-up. Chest x-rays at 6 months and CT scans at 12 months after radiation therapy revealed that response to radiation was maintained, with no evidence of disease progression.
Meanwhile the symptoms of chronic sinusitis and nasal congestion worsened. She was transferred to the department of Otolaryngology/Head and Neck Surgery where a CT scan revealed a mass at the bottom of the left maxillary sinus and the nasopharynx. Surgical excision confirmed amyloid deposits submucosally at the roof of the nasopharynx. Functional endoscopic sinus surgery (FEES) was performed to ventilate the left maxillary sinus. To date no further treatment has been necessary.