Tracheobronchial Amyloidosis Mimicking Tracheal Tumor.

Elif Tanrıverdi,Şule Gül,Murat Acat,Mehmet Akif Özgül,Erdoğan Çetinkaya,Oğuz Uzun,Naciye Arda,Mustafa Çörtük,Zehra Yaşar

doi:10.1155/2016/1084063

Abstract

Tracheobronchial amyloidosis is a rare presentation and accounts for about 1% of benign tumors in this area. The diagnosis of disease is delayed due to nonspecific pulmonary symptoms. Therapeutic approaches are required to control progressive pulmonary symptoms for most of the patients. Herein, we report a case of a 68-year-old man admitted with progressive dyspnea to our institution for further evaluation and management. He was initially diagnosed with and underwent management for bronchial asthma for two years but had persistent symptoms despite optimal medical therapy. Pulmonary computed tomography scan revealed severe endotracheal stenosis. Bronchoscopy was performed and showed endotracheal mass obstructing 70% of the distal trachea and mimicking a neoplastic lesion. The mass was successfully resected by mechanical resection, argon plasma coagulation (APC), and Nd-YAG laser during rigid bronchoscopy. Biopsy materials showed deposits of amorphous material by hematoxylin and eosin staining and these deposits were selectively stained with Congo Red. Although this is a rare clinical condition, this case indicated that carrying out a bronchoscopy in any patient complaining of atypical bronchial symptoms or with uncontrolled asthma is very important.

Highlights

Amyloidosis is characterized by the abnormal extracellular deposition of insoluble fibril proteins
Diseases are detected as systemic amyloidosis or localized amyloidosis
Systemic amyloidosis is classified into four categories as primary, immunoglobulin light chain (AL) disease; secondary, amyloid A protein (AA) disease; hereditary, mutant transthyretin (ATTR) disease; dialysis-dependent, beta 2 microglobulin (β2m) disease

Summary

Introduction

Amyloidosis is characterized by the abnormal extracellular deposition of insoluble fibril proteins. Diseases are detected as systemic amyloidosis or localized amyloidosis. Systemic amyloidosis is classified into four categories as primary, immunoglobulin light chain (AL) disease; secondary, amyloid A protein (AA) disease; hereditary, mutant transthyretin (ATTR) disease; dialysis-dependent, beta 2 microglobulin (β2m) disease. Localized amyloidosis is more common than systemic amyloidosis. Localized amyloidosis is limited single organ such as bladder, skin, heart, or lung [1, 2]. The most cases of respiratory amyloidosis have been described in three forms: diffuse interstitial amyloidosis, nodular parenchymal amyloidosis, and tracheobronchial amyloidosis [3]. With only around one hundred cases reported in the literature, tracheobronchial amyloidosis is a rare form of localized amyloidosis [4]

Case Reports

Findings

Discussion