Organ-Limited Laryngeal Amyloid Deposits: Clinical, Morphological, and Immunohistochemical Results of Five Cases

Abstract

Five cases of organ-limited laryngeal amyloid deposits with no evidence of systemic disease are reported in detail and classified immunohistochemically. In four of the five cases the amyloid reacted with anti-A lambda antibodies and in one case with anti-A kappa antibodies. Four of our five female patients had already passed the fifth decade of life. One was 11 years old. Hoarseness was the predominant symptom in four cases, in which we found amyloid deposits in the glottic area. Only one patient, with amyloid deposits in the aryepiglottic fold, complained of pain. The therapy of choice of idiopathic, localized, or organ-limited amyloid deposits without underlying disease may be local excision. In one of the cases reported in this paper, a laryngofissure was performed, and in another a partial laser resection was performed. No therapy was performed in three of our five cases. In the larynx, as in many other locations and only if possible, removal at intervals is more feasible than radical resection, because these amyloid tumors grow slowly.

Amyloidosis of the Larynx: A Clinicopathologic Study of 11 Cases

“…The literature supports that most cases of amyloidosis of the larynx are composed of a protein that is immunologically identical to the variable region of the light chain fragment of immunoglobulin (1, 7-9, 16, 17, 23, 24) and is classified as a fibril type, similar, if not identical, to that of primary amyloid (2, 3, 5, 7, 16, 24 -27). has been reported with greater frequency, perhaps because light chains have a ␤-pleated configuration similar to amyloid (5,6,8,16). Although immunophenotyping has become universally available, the "gold standard" for the diagnosis of amyloid remains a tissue biopsy demonstrating characteristic hematoxylin and eosin changes and Congo red birefringence or metachromatic pink-violet staining with methyl violet or crystal violet.…”

“…This intimate association of the lymphoplasmacytic infiltration is different from systemic amyloidosis whereby the plasma cells are spatially separated from the amyloid deposition (2-4, 7, 9, 17, 24, 26, 30). Many LA cases in the literature have been associated with immunostaining for light chain restriction of both the plasma cells and the amyloid (1,8,16,17).…”

Amiloidose localizada laríngea: relato de caso e revisão de literatura

“…Ela é mais expressiva nos casos em que se observa ao diagnóstico maior extensão da lesão. No entanto, são raros os casos que evoluem a óbito devido obstrução respiratória ou que apresentam amiloidose sistêmica 2,6,8 .…”

Localized ENT Amyloidosis – Literature Overview

“…Localized amyloid tumors in the head and neck region are an extremely rare manifestation that is not usually associated with either multiple myeloma or systemic amyloidosis (Godbersen GS, 1992).…”

“…Computed tomography scans demonstrate focal amyloidosis as a well-defined, submucosal, homogeneous, hyperdense, soft tissue mass in association with calcifications (Gean-Marton AD, 1991) (Panda NK, 2007) (Pitkäranta A, 2000). The lesions are described as well-defined homogeneous soft-tissue masses, with no or only a slight degree of contrast enhancement (Godbersen GS, 1992). Occasionally, areas of localized calcification within the mass are present (Hegarty JL, 1993) (Parmar H, 2010).…”