BrazJOtorhinolaryngol.2015;81(2):219---221
www.bjorl.org
Brazilian
Journal
of
OTORHINOLARYNGOLOGY
CASE
REPORT
Laryngeal
amyloidosis
presenting
as
false
vocal
fold
bulging:
clinical
and
therapeutic
aspects
夽
Amiloidose
laríngea
apresentando-se
como
abaulamento
em
prega
vestibular:
aspectos
clínicos
e
terapêuticos
José
Caporrino
Neto
a,∗,
Neisa
Santos
Carvalho
Alves
b,
Luiza
de
Almeida
Gondra
baDepartmentofOtorhinolaryngologyandHeadandNeckSurgery,UniversidadeFederaldeSãoPaulo(UNIFESP),
SãoPaulo,SP,Brazil
bEscolaPaulistadeMedicina,UniversidadeFederaldeSãoPaulo(UNIFESP),SãoPaulo,SP,Brazil
Received4September2014;accepted2November2014 Availableonline14January2015
Introduction
Thelarynxisthemainaffectedsiteinupperaerodigestive tractamyloidosis, whichpredominates in menin thefifth decadeoflife1,2andrepresentsapproximately1%ofbenign tumorsinthisorgan.1Dysphoniaisthemainsymptom3,4and histopathologicaldiagnosisisthegoldstandard.
Thisstudydescribesacaseoflaryngealamyloidosisina youngfemalepatient,followedatthisservicefrom Novem-berof2011toOctoberof2013,andaimstodemonstratethe clinicaldiversityofthedisease,aswellastodrawattention tothedifferentialdiagnosisandfollow-up.
Case
presentation
A35-year-oldblackfemalepatient,bornandliving inSão Paulo,SP,Brazil,complainedofodynophagia(worseonthe
夽 Pleasecitethisarticleas:CaporrinoNetoJ,AlvesNS,GondraLA.
Laryngealamyloidosispresentingasfalsevocalfoldbulging:clinical andtherapeuticaspects.BrazJOtorhinolaryngol.2015;81:219---21.
∗Correspondingauthor.
E-mail:jose.capo.neto@gmail.com(J.CaporrinoNeto).
right),throatclearing,globuspharyngeus,anddysphoniafor fouryears.She deniedrespiratory,gastric, ornasal symp-toms.Sheunderwenttreatmentwithomeprazole40mg/day foranextendedperiod,butshowednosymptom improve-ment.Shedeniedsmoking,alcoholism,orrelevantpersonal andfamilyhistory.
Shepresentedwitharoughvoice;anteriororoscopyand rhinoscopyshowednoalterations.Telelaryngoscopyshowed evidenceofsubmucosalbulginginrightvestibularfold,with no alterations in mobility, and edema/hyperemia of the interarytenoidspace(Fig.1A).
Thehypothesisofasaccularcystwasraised,withlesion excisionfollowedbypostoperativespeechtherapy recom-mended:thesewereperformedwithoutcomplications.
Histopathologicalanalysisresultwaslaryngeal amyloid-osis (Fig. 1B and C) and, therefore, renal and hepatic function tests were requested, as well as an electrocar-diogram, which showed normal results. The patient was referredforfollow-upattherheumatologydepartment.
The patient did not undergo otorhinolaryngology/ rheumatology follow-up and came to the clinic one year aftersurgery, asymptomatic.Newrenal,hepatic,and car-diovascularfunctiontests,aswellasrigidtelelaryngoscopy wereperformed;theresultswerenormal.Semi-annual out-patientfollow-upwasindicatedandpatientwasonceagain referredtotherheumatologydepartment.
http://dx.doi.org/10.1016/j.bjorl.2015.01.001
220 CaporrinoNetoJetal.
Figure1 (A)Telelaryngoscopy:bulgingintherightvestibularfold,withoutglotticorsubglotticinvolvement.(B)Fragmentshowing respiratory-likeepitheliumwithmildlymphomononuclearinfiltrateinthestromaandapparentdepositionofamorphouseosinophilic materialathematoxylin-eosinstaining,intheoutlinedspace(H&E,200×).(C)SameareashowninBwithpalerappearancethan
therestofthesample,whenstainedwithCongored(Congored,400×).
Discussion
Laryngeal involvement usually results from the localized formofamyloidosis4and,contrarytowhatoccursinother headandnecksites,itisrarelyinvolvedinsystemicdisease cases.3
Thelesionoccursmainlyinthevestibularfold(55%), pre-sentingassubepithelialedemaor nodularformation.1 The diagnosisisrarelysuspectedandisoftenonlyattainedafter anatomopathologicalanalysis,which disclosestypical pos-itive birefringence under polarized light after Congo red staining, showing a greenish color.5 In the present case, asaccular cystwassuspected,a diseasecharacterizedby obstruction/atresia of the laryngeal saccule orifice, with consequentmucusretention andsubmucosal bulging. Sar-coidosis,metastatictumors,polyps,malignantneoplasms, and salivary gland tumors are other possible differential diagnoses.4,6
Someauthorssuggesttheassessmentofhepaticandrenal functions, as well as electrocardiography and endoscopic assessmentofmultifocalamyloidosisoftherespiratorytract in the initial investigation of systemic involvement.4 The normal results of these tests in this patient, in addition tothe asymptomaticpostoperativecourse, suggesta case oflocalizedamyloidosis.However,theimportanceofafull rheumatological assessment in these patients is notewor-thy.
Treatmentvariesfromobservationtosurgicalexcision.3 Immunosuppressive agents and radiotherapy have shown to be ineffective and can accelerate the amyloid deposition.5 In localized and symptomatic laryngeal
pictures, endoscopic surgical excision is the treatment of choice.3 In contrast, in extensive cases without immi-nent airway obstruction, the expectant conduct can be chosen, considering the slowly progressive nature of the disease.2
Prognosis is excellent and the long-term follow-up should be conducted for at least five to seven years, due to the possibility of late recurrence and systemic involvement.4,5
Final
comments
Laryngeal amyloidosis is a disease whose diagnostic sus-picion is difficult, considering its clinical diversity and similarities to other more relevant diseases in the pop-ulation. The prognosis is excellent; however, long-term follow-upshouldbeperformed,duetothepossibilityoflate recurrenceofthediseaseandsystemicinvolvement.
Conflicts
of
interest
Theauthorsdeclarenoconflictsofinterest.
References
Laryngealamyloidosispresentingasfalsevocalfoldbulging 221
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