Endoscopic management of adult-type rhabdomyoma of the glottis: case report and review of the literature

ARTICLE IN PRESS

Brazilian

Journal

of

OTORHINOLARYNGOLOGY

CASE

REPORT

Endoscopic

management

of

adult-type

rhabdomyoma

of

the

glottis:

case

report

and

review

of

the

literature

夽

Tratamento

endoscópico

do

rabdomioma

glótico

do

tipo

adulto:

relato

de

caso

Filippo

Carta

_,

_Sara

_Sionis

_,

_Clara

_Gerosa

_,

_Roberto

_Puxeddu

a_{DepartmentofOtorhinolaryngology,UniversityofCagliari,SchoolofMedicine,AziendaOspedaliero-Universitaria,}

P.O.SGiovannidiDio,Cagliari,Italy

b_{DepartmentofPathology,UniversityofCagliari,SchoolofMedicine,AziendaOspedaliero-Universitaria,P.O.SGiovannidiDio,}

Cagliari,Italy

Received12February2015;accepted7April2015

Introduction

Rhabdomyomasarebenignmesenchymaltumorscomposed ofstriatedmatureskeletalmusclecells,beingnomorethan 2%of allstriatedmuscletumors,1_{distinguishedin cardiac}

and extracardiac subtypes. Cardiac rhabdomyomas occur

generally in children and are considered hamartomatous

lesions,oftenassociatedwithphacomatoses,suchas

tuber-ous sclerosis,1,2 _{and hamartomas of the kidney and other}

organs.1_{Extracardiacrhabdomyomasareclinicallyand}

mor-phologicallysubdividedinthreesubtypes:thevaginal,fetal

and adult variants. The vaginal-type is a rare tumor-like

polypoid mass, found in the vagina and vulva of

middle-agedwomen.Thefetal-type,withthesubordinatedjuvenile

rhabdomyoma,3_{isprevalentinheadandneckareasin}

chil-dren. Adultextracardiacrhabdomyomaspresent generally

asunifocalheadandnecktumorsinmiddle-agedpatients,4,5

multifocalin14---26%ofcases.6_{Adultrhabdomyomasoccur}

夽 _{Pleasecitethisarticleas:CartaF,SionisS,GerosaC,PuxedduR.}

Endoscopicmanagementofadult-typerhabdomyomaoftheglottis: casereportandreviewoftheliterature.BrazJOtorhinolaryngol. 2015.http://dx.doi.org/10.1016/j.bjorl.2015.04.008

∗_{Correspondingauthor.}

E-mails:filippocarta@unica.it,pippocarta@tiscali.it(CartaF.).

inthe soft tissues of the headand neck up to70---93% of

cases,1 _{while glottic lesionsare extremely rare,and only}

22caseshavebeenreporteduptonow.Withthisarticlewe

reportanadditionalcaseofglotticadult-typerhabdomyoma

andreviewthepertinentliterature,withtwoaims:(I)assess

thestandardofcareofthispathology,toavoidinadequate

treatmentand(II) increase itsknowledgeamongsurgeons

andpathologists.

Case

report

A 75-year-old malewas referred toour department with

a 4-year history of progressive dysphonia. Flexible scope

examinationshowed asmooth submucosalswellingof the

middlethirdoftherightvocalcord,associatedwith

impair-mentofvocal cordmobility.Contrast-enhancedcomputed

tomography (CT) of the neck showed a deep right vocal

cordlesionextendedtotheanteriorparaglotticspace,with

lowanduniformpathologicenhancement(Fig.1).Clinical

andradiologicalfeaturessuggested itsbenign natureand,

therefore, conservativesurgery wasplanned. The patient

underwent transoral CO2 laser excision under general

anesthesiawithCO2 laser(Digital AcuBladeTM,LumenisTM,

Israel)set on 10 Watts, continuous wave in Super-Pulsed

mode/emission, Acu-Blade 2mm of length, under

micro-scopicvision(focallengthof400mm),throughamicroflap

http://dx.doi.org/10.1016/j.bjorl.2015.04.008

1808-8694/©2015Associac¸ãoBrasileirade OtorrinolaringologiaeCirurgiaCérvico-Facial.Published byElsevierEditoraLtda.Allrights reserved.

ARTICLE IN PRESS

2 CartaFetal.

Figure1 Contrast-enhancedcomputedtomography demon-stratesanenhancingrightlaryngealmassdeeplylocatedinthe vocalismuscle.

Figure 2 Laryngeal rhabdomyoma after excision: 22mm×15mm×9mm.

techniqueleavingthemucosaofthevocalcordintact.The

tumor,deeply situatedintotheright vocal cord,was

eas-ily‘‘enbloc’’enucleated andappearedasan ovalnodule

of22mmingreatestdimension(Fig.2).Aftertheexcision,

theminusintotherightthyroaritenoidmuscle(Fig.3)was

leftto heal by secondary intention. Postoperative course

was uneventful: the patient was discharged 1 day after

surgeryandheregainednormalvocalcordmobilityand

nor-malvoicewithin4weeks.Athistology,typicalmorphologic

featuresofadultrhabdomyomawithsheetsoflarge

polygo-nalcellsseparatedbyfewconnectivetissueswerepresent.

Figure3 Endoscopicviewaftertheremoval.

Figure4 Indirectlaryngoscopyat12monthsaftersurgery.

Thecellshadabundanteosinophiliccytoplasmwith

eccen-tricallyplaced nuclei, whereasin some areascytoplasmic

vacuolization with a centrallyplaced nucleus wasfound.

Immunohistochemistryshowedthecellstobestrongly

pos-itive to skeletric muscle actin and desmin. At 12-month

follow-up,thecompleteclosureoftheminuswasobserved

(Fig.4),withnoevidenceofrecurrence.

Discussion

Extracardiac adult and fetal types rhabdomyomas

proba-bly originatefromskeletalmuscle ofthe thirdandfourth

branchial arches.1,7 _{Theirneoplastic naturewasnot clear}

because tumor cells usually do not express cell

prolifer-ation markers such as Ki-67 and PCNA, resembling more

likely hamartomas than neoplasms.7 _{In 1992, Gibas and}

Miettinemdemonstratedfewchromosomalclonalanomalies

supportingtheneoplasticnatureofrhabdomyomas.8_Before

this case,22 casesof adult-typelaryngeal rhabdomyomas

havebeen reported(Table1):Johansenandcoworkers,in

1995,reviewedallcasesofadultrhabdomyomasofthe

lar-ynx (n=12) previously described1_{; after 1995, 10 further}

caseshavebeenpublished.Agerangesfrom16-yearoldto

79-yearold(meanage59years,59%ofpatientsinthesixth

andseventhdecades, sexratioM/Fof 1:1.75);the tumor

was found in the glottis in 12 cases, in the arytenoid in

4 patients and in the supraglottis in 7 patients; although

stridor and airway obstruction can develop abruptly, the

lesiongenerallyremainsasymptomatic,untilitcauses

symp-toms like dysphonia (86%), dysphagia (18%) and dyspnea

(18%), that usuallyprogress slowly (median duration-time

of 2.5 years) (Table 1). Macroscopic appearance is

usu-ally a submucosal swelling with possible deep extension

inside the laryngeal framework, but they may be

ses-sile.Differential diagnoses includeneurogenic or vascular

tumors, oncocytoma, osteoma, Abrikossoff’s tumor and

rhabdomyomasarcoma.1 _{Radiographically adult}

rhabdomy-omapresentsasanhomogenouslesion,isointenseorslightly

hyperintensetomuscleonT1-aswellasT2-weighted MRI

andslightlyhyperdenseonCT.4 _{Athistology,theadultand}

thefetaltypehavetobedistinguished:theformerclosely

ARTICLE IN PRESS

EndoscopiclaserCO2managementofadult-typelaryngealrhabdomyoma 3

Table1 Adult-typelaryngealrhabdomyomas.

Source(year) Location Age/sex ChiefComplaint/

durationofsymptoms

Treatment Comment

Climeetal.(1963) Vocalcord 48/M Hoarseness/3months Endoscopic

excision

Norecurrence reported

Battiforaetal.(1969) Glottis 55/M Hoarseness/3years Excisionwith

laryngofissure

Nofollow-upreported BianchiandMuratti

(1975)

Rightfalsevocal cord

52/F Hoarseness Endoscopic

excision

Norecurrence reported Bagbyetal.(1976) Rightfalsevocal

cord

55/M --- Endoscopic

excision

Norecurrence reported Ebbesenetal.(1976) Rightventricle 64/F Hoarsenessandforeign

bodysensation/6months

Endoscopic excision

Norecurrence reported

Winther(1976) Vocalcord 39/M Hoarseness/3years Endoscopic

excision

Recurrence BoedtsandMestdagh

(1979)

Vocalcord 76/F Hoarseness/2months Endoscopic

excision

Norecurrence reported KleinsasserandGlanz

(1979)

Glottis 16/M Acuteairway

obstruction/sudden onset Total laryngectomy Initialmisdiagnosisof Rhabdomyosarcoma Helliwelletal.(1988) Leftvocalcord 52/M Hoarseness/6months Excisionwith

lateral pharyngotomy

Norecurrence reported

Heliwelletal.(1988) Rightvocalcord 66/M Hoarseness/8years ? Nofollow-upreported

Hamperetal.(1989) Arytenoid 51/F Dyspneaanddysphagia ? Recurrence

Johansenetal.(1992) Leftventricule 51/M Hoarseness,snoring/1 year

Hemilaryngectomy Norecurrence reported

Selmeetal.(1994) Vocalcord 31/F Hoarseness Completeremoval

afterendoscopic biopsy Clonalchromosomal anomalies LaBagnaraetal. (1999)

Vocalcord 69/F Hoarseness/5years Endoscopic

excision

Restaurationof normalvocalcord functionwithin6 months

Orritetal.(2000) Arytenoid 66/M Hoarsenessand

dysphagia/4months

Externalremoval Vocalcordpalsy Brysetal.(2005) Rightfalsevocal

cord

79/M Hoarseness/5years Externalremoval Dischargedafter10 daysfromthe hospital

Liessetal.(2005) Epiglottis 69/M Asymptomatic --- Multifocal

JensenandSwartz (2006)

Rightarytenoid 66/M Dysphagia,hoarseness/3 yearsandsudden dyspnea Endoscopic excision Desminhigh reactivity. 18monthoffollow-up Koutsimpelasetal. (2008) Left aryepiglotticfold 72/F Globulusand hoarseness/1year Endoscopic excision Multifocallesion Farboudetal.(2009) Arytenoid 76/M Hoarsness,dysphagia

andsleep-apnoea Tracheostomyand endoscopic multipledebulking procedures Bilateral

Friedman(2012) Glottis --- Dysphonia Endoscopic

excision

---Cainetal.(2013) Supraglottis 67/F Hoarsenessand

progressivedyspnea Tracheotomyand hemilaryngectomy At16months completeglottic closurewith phonationandno evidenceof recurrence

Presentcase(2013) Rightvocalcord 75/M Hoarsness/4years Endoscopic

excision

ARTICLE IN PRESS

4 CartaFetal.

cells with PAS-positive granular or vacuolated cytoplasm, while the fetal typeis composed withless differentiated neoplasticcells.3 _{Immunohistochemistrydemonstratesthe}

muscleimmunophenotype,withstrongpositivityformuscle

specificmarkers;inourcaseandintheliterature,desmin

appearedasareliablemarker.1,2

Definitive treatment for laryngeal adult rhabdomyoma

is complete excision; although extensive lesions reported

intheliterature requiredin 8casesan external approach

(Table 1), including a total laryngectomy, when glottic

rhabdomyomaisconfinedtotheendolarynx,thetransoral

approach should be preferred. Transoral minimally

inva-sive laser CO2 assisted excision appears to be optimal in

terms of efficacy and low morbidity: the vocalis muscle

andthemucosacan beonlyincised withoutany removal.

Sincededifferentiationofanadultrhabdomyomatoa

malig-nantvarietyis notdocumented,amoreinvasiveapproach

may appear an overtreatment, but a radical excision is

mandatorysince recurrences are possible (2 cases in the

literature),9,10 _{attributabletoincompleteprimaryexcision,}

thatcanoccursincetheconsistenceofthelesionisfriable.

Conclusion

Laryngealrhabdomyomaisararebenigntumorthathasto

beconsideredinthedifferentialdiagnosisofallsubmucosal

laryngeallesions.Conservativeapproachisadvisablesince

thetumorcanbeendoscopicallyenucleated.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

Acknowledgments

TheauthorsgratefullyacknowledgeSardiniaRegional

Gov-ernment for the financial support (P.O.R. Sardegna F.S.E.

Operational Programmeof theAutonomousRegionof

Sar-dinia, European Social Fund 2007---2013 --- Axis IV Human

Resources, Objectivel.3, Line of Activityl.3.1 ‘‘Avvisodi

chiamataperilfinanziamentodiAssegnidiRicerca’’).

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2.FaviaG,LoMuzioL,SerpicoR,MaioranoE.Rhabdomyomaofthe headandneck:clinicopathologicfeaturesoftwocases.Head Neck.2003;25:700---4.

3.SharmaSJ,KreiselM,KrollT,GattenloehnerS,KlussmannJP, WittekindtC.Extracardiacjuvenile rhabdomyomaofthe lar-ynx: a rare pathological finding. EurArch Otorhinolaryngol. 2013;270:773---6.

4.DeTreyLA,SchmidS,HuberGF.Multifocaladultrhabdomyoma oftheheadandneckmanifestationin7locationsandreviewof theliterature.CaseRepOtolaryngol.2013;7584:16.

5.BrysAK,SakaiO,DeRosaJ,ShapshaySM.Rhabdomyomaofthe larynx:casereportandclinicalandpathologicreview.EarNose ThroatJ.2005;84:437---40.

6.OrritJM,RomeroC,MallofréC,TraserraJ.Laryngeal rhabdomy-oma:unusualcaseofdysphonia:reviewoftheliterature.Acta OtorrinolaringolEsp.2000;51:643---5.

7.Maglio R, Francesco S, Paolo M, Stefano V, Francesco D, GiovanniR. Voluminous extracardiac adult rhabdomyoma of the neck: a case presentation. Case Rep Surg. 2012;2012: 984789.

8.GibasZ,MiettinenM.Recurrentparapharyngealrhabdomyoma: evidenceofneoplasticnatureofthetumorfromcytogenetic study.AmJSurgPathol.1992;16:721---8.

9.Hamper K, Renninghoff J, Schäfer H. Rhabdomyoma of the larynx recurring after 12 years: immunocytochemistry and differential diagnosis. Arch Otorhinolaryngol. 1989;246: 222---6.

10.Winther LK. Rhabdomyoma of the hypopharynx and larynx: reportoftwocasesandareviewoftheliterature.JLaryngol Otol.1976;90:1041---51.