Endoscopic management of laryngo-tracheobronchial amyloidosis: a series of 32 patients

Abstract

Between June 1983 and December 2002, 32 patients were treated for primary localized laryngo-tracheobronchial amyloidosis (LTBA) at our institution. For enrollment in this retrospective study, at least one positive biopsy with Congo red stain and a diagnostic test battery excluding systemic or secondary amyloidosis were mandatory. The most common presenting symptom was dyspnea, which was observed in 75% of cases. Endoscopic appearance was described as submucosal plaques and nodules with a cobblestone appearance in 44% of patients, tumor-like in 28% and circumferential wall thickening in 28% of cases. Tracheobronchopatia osteochondroplastica was associated with LTBA in 22% of patients, both at the first treatment (four cases) or during follow-up examinations (three). Synchronous or metachronous disease in the larynx and tracheobronchial tree was observed in 47% of cases, while only one patient showed lung involvement with extensive amyloidosis of the trachea and bronchi. All but two patients were endoscopically treated. One of these was affected by a tracheal amyloidoma cured by endoscopic debulking and further tracheal resection-anastomosis. The other patient presented massive laryngo-pharyngeal involvement and was therefore treated by total laryngectomy. A total of 58 endoscopic procedures (range: 1-7; mean: two per patient) were carried out, 11 with CO2 laser (for supraglottic, glottic and selected subglottic lesions) and 47 with Nd:YAG laser (for the most part of subglottic lesions and amyloid deposits located in trachea and bronchi). Four patients required a tracheotomy and three necessitated a T-tube stent during management, which was subsequently removed in all cases. One patient was lost to follow-up, nine were endoscopically controlled elsewhere (Group A) and 22 were followed at our institution (Group B). In Group A, the patient with tracheal amyloidoma is asymptomatic and free of additional LTBA localizations 8 years after surgery. The remaining eight are asymptomatic, but with persistent endoscopic signs of amyloidosis. In Group B, five patients died: one from heart failure and another one from pneumonia 3 and 4 days after surgery, respectively. In one case death was from unrelated causes, and in the two remaining patients it was from respiratory failure due to uncontrolled bilateral bronchial amyloidosis in the subsequent 5 to 6 years. One patient is completely cured 5 years after surgery, and 16 are asymptomatic with persistence of LTBA.

Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): A consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis

“…Amyloid goiter may also be seen in secondary amyloidosis (AA) [24]. Amyloidosis of the tracheobronchial tree and larynx [25], although a potentially serious condition, is not a component of a systemic amyloidosis syndrome. The finding of amyloid deposits in the cardiac atria [26], pleura, and articular cartilage [27,28] may also represent localized amyloid, and additional evidence of visceral involvement is required before systemic amyloidosis is diagnosed.…”

Pulmonary Manifestations of Amyloidosis

“…Tracheobronchial amyloidosis was associated with TBO in 22% in one series. (Piazza et al, 2003) Ultimately, the diagnosis of pulmonary amyloidosis requires histologic confirmation. An excellent overview of the pathologic features of pulmonary amyloidosis has been published.…”

“…In a retrospective series of 32 patients over 19 years, sixteen had persistent asymptomatic endolumenal disease. (Piazza et al, 2003) Two patients had endolumenal procedures with subsequent requirement of surgical resection. Each patient had no evidence of recurrent endolumenal amyloid after 5 to 8 years.…”

Localized Amyloidosis of the Head and Neck

“…In a pseudotumor mass or a circumferential wall thickening, mechanical resection and/or dilatation coupled with transmucosal Nd:YAG laser treatment with low power and high energy density are recommended. On the other hand, submucosal plaques, particularly when located at the glottic or subglottic levels, should be treated by a mucosa-sparing approach or by CO 2 laser, thus minimizing scar tissue formation and subsequent complications (Piazza et al, 2003). Localized laryngeal amyloidosis is a slowly progressive disease that should be treated according to the complaints of the patient.…”