Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Primary Pulmonary Amyloidosis
- Radiologic Findings
- The abnormality in tracheobronchial tree was incidentally detected on the Chest CT which was performed to evaluate the breast cancer.
With the suspicion of tracheobronchial amyloidosis, the patient underwent bronchoscopy.
Diffuse mucosal swelling and luminal narrowing was noted and the fixed obstruction of bronchus which did not dilate with inspiration was observed. Mucosal biopsy was done at bilateral proximal bronchus. The pathology revealed chronic inflammation with noneoplastic bronchial tissue and Congo-red staining revealed amyloid of greenish birefringence under polarized light microscopy.
- Brief Review
- Primary pulmonary amyloidosis is a rare disorder that appears in three forms: tracheobronchial form, nodular parenchymal form, and diffuse parenchymal form. Primary tracheobronchial amyloidosis is generally not associated with parenchymal abnormalities. Amyloid materials deposit in the submucosal area and most commonly involve the entire trachea. Infrequently, a solitary submucosal nodule can be present. On CT, tracheobronchial amyloidosis usually leads to diffuse, concentric, smooth, or nodular thickening of the submucosal tracheal wall. Cartilage is normal but concentric calcification or ossification may occur without tracheomalacia. Bronchoscopy proved to be the most useful diagnostic method in establishing the diagnosis by biopsy. Diagnosis requires histological confirmation through biopsy, in which pathologic specimens stained with Congo-red reveal greenish birefringence under polarized light microscopy.
- References
- 1. Kim H.Y., Im J.G., Song K.S., et al. Localized amyloidosis of the respiratory system: CT features. J Comput Assist Tomogr 1999;23:627-631.
2. O'Regan A., Fenlon H.M., Beamis J.F., Jr, et al. Tracheobronchial amyloidosis. The Boston University experience from 1984 to 1999. Medicine (Baltimore) 2000;79:69-79
- Keywords
- Airway, Metabolic and storage lung disesae,