Localized Amyloidosis of the Head and Neck and Upper Aerodigestive and Lower Respiratory Tracts

Abstract

Seven cases of localized amyloidosis limited to structures of the head and neck and upper aerodigestive and lower respiratory tracts evaluated and treated at Boston University Hospitals in a recent 7-year period were reviewed. Negative Congo red staining of abdominal adipose aspiration biopsy or rectal biopsy specimens established that the amyloidosis was not systemic. Localized amyloidosis occurred in discrete masses in a variety of sites in the aerodigestive tract including the orbit, nasopharynx, lips, floor of mouth, tongue, larynx, and tracheobronchial tree. Five patients required surgical excision because of significant airway obstruction or organic dysfunction. Amyloid deposits completely excised with the carbon dioxide laser have not recurred, though other amyloid masses may appear elsewhere within the same organ or region. Amyloidosis may occur primarily or secondarily to other disease states. Localized amyloidosis has not been chemically identified but is usually defined by the absence of systemic features. While rare, amyloidosis must be recognized and understood by the otolaryngologist/head and neck surgeon to allow appropriate diagnostic and therapeutic planning.

Localized Amyloidosis in the Nasopharynx and Neck: Case Report

“…3 Simp son et al ha ve re por ted less re cur ren ce fol lo wing la ser ex ci si on of lo ca li zed amy lo i do sis. 5 Lo ca li zed amy lo i do sis is a di se a se of gre at ra rity and slow prog res si on, for which sponta ne o us reg res si on is known to oc cur; the re fo re, it is dif fi cult to pre dict the out co me. 6 Re cur rent masses and ex ten si ve le si ons in vol ving the he ad and neck sho uld be hand led con ser va ti vely.…”

“…thre e dis tinct forms: AL amy lo i do sis, as so ci a ted with pri mary syste mic amy lo i do sis; AA se con dary amy lo i do sis as so ci a ted with ne op las tic or inf lamma tory con di ti ons; and fa mi li al amy lo i do sis, which shows re ces si ve in he ri tan ce. 5 Lo ca li zed amy lo i do sis in the he ad and neck is a ra re and be nign pro cess. The most com mon si tes of in vol ve ment are the larynx, subg lot tis and thyro id.…”

Amiloidose com múltiplos focos em trato aéreo-digestivo superior: relato de caso e revisão de literatura

“…Beiser 6 described one case of amyloidosis in the tongue base, tonsils and rhinopharynx. Simpson described a case of amyloidosis in the rhinopharynx only, causing Eustachian Tube dysfunction 13 . Green 14 reported recurrent amyloidosis in many distinct laryngeal sites, such as the tonsils, tongue base, rhinopharynx and supraglottis.…”

A Rare Sinonasal Entity

“…The lack of enhancement may help differentiate this process from cellular tumors. 1,6 Amyloidosis must be confirmed by biopsy and pathologic analysis. This is achieved through Congo red staining, which demonstrates a characteristic "apple green" birefringence visible under polarized microscopy.…”