Focus on localized laryngeal amyloidosis: management of five cases

Abstract

Amyloidosis is a group of idiopathic clinical syndromes caused by the deposition of insoluble fibrillar proteins (amyloid) in the extracellular matrix of organs and tissues. These deposits disrupt the function of the target organ. Amyloidosis can manifest as a systemic disease or a single-organ involvement (local form). Its etiology still remains unclear. Deposits of amyloid in the larynx are rare, accounting for between 0.2 and 1.2% of benign tumors of the larynx. In this retrospective study, we report the clinical aspects, diagnosis, treatment and follow-up of five female patients with localized laryngeal amyloidosis without systemic involvement. The patients were all treated successfully using microlaryngoscopy with CO₂ laser or cold instruments. Prognosis is excellent; however, appropriate follow-up is an important part of the long-term management of this disease in order to prevent and control the possibility of local recurrence.

Figure 1

Histological preparation. (A) Excisional biopsy of the left vocal cord reveals a deposit of amorphous eosinophilic material in the tissue, consisting of amyloid (hematoxylin–eosin stain). Magnification 4×. (B) Detailed image of amyloid deposits. Magnification 10×. (C and D) Deposits are highlighted on Congo red stain. Magnification 10×.

Figure 2

Case 5: endoscopic image before (A) and after (B) the surgical excision. A small formation in correspondence of the surgical excision at 4-month laryngeal control (C). Last follow-up examination (D).

Figure 3

Intraoperative images: before (A) and during (B) the surgical excision.