Esthesioneuroblastoma located in the thoracic extradural space: Case report

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ContentslistsavailableatScienceDirect

International

Journal

of

Surgery

Case

Reports

jo u r n al ho me p a g e :w w w . c a s e r e p o r t s . c o m

Esthesioneuroblastoma

located

in

the

thoracic

extradural

space:

Case

report

Mehmet

Hüseyin

Akgul

(MD)

(Assistant

Professor)

_,

_Ferruh

_Gezen

_(PhD,MD)

_,

Ali

Kemal

Uzunlar

(PhD,MD)

a_{KırıkkaleUniversityMedicalFaculty,DepartmentofNeurosurgery,Kirikkale,Turkey} b_{MedeniyetUniversityMedicalFacultyDepartmentofNeurosurgery,Istanbul,Turkey} c_{DuzceUniversityMedicalFaculty,DepartmentofPathology,Düzce,Turkey}

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f

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Received5April2016

Receivedinrevisedform26May2016 Accepted8June2016

Availableonline30July2016 Keywords:

Esthesioneuroblastoma Methastasis

Thoracalvertebrae

a

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OBJECTIVE:Esthesioneuroblastomaaccountedforonly6%ofthemalignantnasalcavityneoplasms(ENB) isararetumorwhichoriginatesfromtheolfactoryepithelium.ENB’sarelocallyagresiveandcan metas-tasizebylymphaticandhematogenousroutes.Apatientwiththemassonthenasaldorsumwasreported inthisarticle.

CASEHISTORY:A52–year-old-manadmittedtothehospitalwitha3monthshistoryofprogressivenasal obstruction,epistaxisandmassonthenasaldorsum.Onrhinoscopy,apolypoidmasswasseeninthe bothnasalcavityandintranasalbiopsywithlocalanesthesiawasperformed.Histopathologicdiagnosisof thetumorwasKadishstageBesthesioneuroblastoma.Tumorwasexcisedbyusingbilateralendoscopic endonasalresectionandlateralrhinotomyapproachandparanasalradiotherapyperformed postopera-tively.Tenmonthsaftersurgery,neckmetastasiswasoccuredandpatientwasunderwentneckdissection. Twenteethmonthsafterinitialtreatment,distantmetastasiswasidentifiedontheT10vertebraand fol-lowingthecranialandspinalradiotherapytotheneckhewasfreeoflocalrecurrenceatfollowup13 monthsaftersurgery.

CONCLUSION:IthasbeenknownthatthemetastasisoftheENBtothespinalcordisanuncommonevent, anditoccursoftenyearsafterinitialdiagnosis.MRIscanishelpfulformakingthediagnosis,andsurgery isthetreatmentofchoiceforobtainingdiagnostictissueanddebulkingthetumor.Radiotherapyisalso amainstayofpostoperativetreatment.

©2016PublishedbyElsevierLtdonbehalfofIJSPublishingGroupLtd.Thisisanopenaccessarticle undertheCCBY-NC-NDlicense(http://creativecommons.org/licenses/by-nc-nd/4.0/).

1. Introduction

Esthesioneuroblastoma (ENB) accounted for only 6% of the malignantnasalcavityneoplasms(ENB)isararetumorwhich orig-inatesfromtheolfactoryepithelium[1].Althoughthesetumors haveabimodalagedistributionoccuringsecondandsixthdecades oflife,itcanbeseenallagegroups[2].ENBsarelocallyagresive andcanmetastasizebylymphaticandhematogenousroutes.The cervicallymphnodesarethemostcommonsiteofmetastasis.It canspreadsubmucosallyinalldirections,involvingtheparanasal sinuses,nasalcavitiesandcrossthecribriformplateandinvolving brain.Becauseofnonspesificsemptomssuchasnasalobstruction, epistaxis,headache,diagnosisfrequentlydelayed. ˙Inthisarticlewe reporteda caseofdiagnosisdelayedpatientattendedourcilinc afteroccurthemassonthenasaldorsum.

∗ Correspondingauthor.

E-mailaddresses:drmhakgul@yahoo.com(M.H.Akgul),

ferruhgezen@duzce.edu.tr(F.Gezen),alikemaluzunlar@gmail.com(A.K.Uzunlar).

2. Casereport

A52–year-oldmanadmittedtotheEar-Nose-Troathclinicwith history ofprogressive nasalobstruction, andepistaxis during3 months.Onrhinoscopy,apainlesspolypoidmassin3×3cm diam-eters was seen in the both nasal cavity and thenasal dorsum withoutpalpablmassontheneck.Hisvisionandeyemovements werenormalinbotheyes.Aparanasalcomputedtomography(CT) scanconfirmedthis massfilledboth nasalcavity. Althoughthis massextendedintothebilateralethmoidandfrontalsinusesand invadedleft laminapaprisea,its intracranialextension wasnot seenonCTimages. Intranasalbiyopsiwithlocalanesthesia was performedtothemass,andhistopathologicaldiagnosiswasENB withkadishstageB. Themasswasremovedneartotalthrough thebilateralendoscopicendonasalapproachandlateralrhinotomy approach. Whole paranasal radiotherapy was performed post-operatively.Uneventfully,neckmetastasisofresidualmass was identifiedtenmonthsaftersurgery,andwholeneckradiotherapy protocolwasperformedtothepatient.Afterradiotherapy,hewas

http://dx.doi.org/10.1016/j.ijscr.2016.06.015

2210-2612/©2016PublishedbyElsevierLtdonbehalfofIJSPublishingGroupLtd.ThisisanopenaccessarticleundertheCCBY-NC-NDlicense(http://creativecommons. org/licenses/by-nc-nd/4.0/).

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M.H.Akguletal./InternationalJournalofSurgeryCaseReports27(2016)70–73 71

Fig.1.A.T1thoracalsagittalMRIshowsepiduralmetastaticENBinT9-10level.B.T2thoracalsagittalMRIshowsepiduralmetastaticENBinT9-10level,C.T1thoracal sagittalcontrast-enhancedMRIshowingintensehomogeneousenhancementofepiduralmetastaticENBinT9-10level,andD.T1thoracalsagittalcontrast-enhancedMRI showingintensehomogeneousenhancementofepiduralmetastaticENBintheleftsidetheT9-10levelofepiduralspace.

underwenttotheextensiveneckdissectionsurgery.

histopatho-logicaldiagnosiswassame.

Twomonthsafterthesecondsurgery,hewasadmittedtothe

hospital withacute paraplegia.Sensory exam was unfeelingto

lighttouchandpinprickoflowerdermatomastoT12with

are-flexi.Babinskireflexwasplantarextensorinrightlegandflexorin

leftleg.Analtonusitewasmild.Hiscompletebloodchemistrywas

withinnormallimits.Anemergencythoracalmagneticresonance

imaging(MRI)withgadoliniumshowedanenhancingextradural

tumorwhichalmostcompletelyfilledthespinalcanaland

tho-racal10 (T10) vertebrae body. Otherorganmetastasis wasnot

foundradiologically(Fig.1).Thepatientwasimmediatelytaken

tooperationroom.TotallaminectomywasperformedtotheT10 vertebrae.Then, largeextraduralmass wasremovedsubtotally. ThehistopathologicaldiagnosiswasENB(Fig.2).Oneweekafter laminectomy,T9andT10anteriorcorpectomy,T10-11discectomy andT9-T10-T11cage-plaque-screw-linkstabilizationthroughthe right toracotomy was performed tothe patient to remove the tumoralmassgrosstotallyandtoprotecthimfromthe progres-sivekyphoticdeformity.Thepostoperativeperiodwasuneventful, and hisneurological examination wasbetterthan preoperative

period.Hewasdischargedtohomeon14thdaypostoperatively. Hewasdiedoflocalrecurrenceatfollowup13monthsafterthe lastsurgery.

3. Discussion

ENBisararemalignanttumorofnasalcavity,anditarisesfrom theolfactoryneuroepitheliumlocatedinthenasalseptum, cribri-formplateandthemiddleandsuperiorturbinates[3].Commonly, ENBcausesunilateralnasalobstructionandepistaxis.Italsocauses anosmia,headache,proptosis,diplopiaandexcessivelacrimation whentumorextendstotheorbita.Inpresentcase,thepatient com-plainedofbilateralnasalobstruction,epistaxisduetothetumor locatedinthebothnasalcavityandthenasaldorsum.

ENBs are staged clinically with kadishsystembased on the spreadofthetumor[2].Accordingtothissystem,

-stageAtumorsareconfinedtothenasalcavity, -stageBlesionsinvolvethesinuses,and

-stageCmassesinvolvethemiddlecranialfossaandthe retrobul-barorbit[4].

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Fig.2.HistologicsectionsofT9-10preoperativelesionconfirmtheimpressionofametastaticENB.Thetumoriscellularandispositiveformarkersofneuronal(neuron specificenolase).Aswellasofasustentaculardifferentiation.Originalmagnification:×200.

Table1

Cedars-Sinaiclassificationsystemformetastaticesthesioneuroblastomatothespine.

Stage Anatomiclocationoflesion

CS0-leptomeningealdisease InvolvementoftheCSFandleptomeninges(arachnoidandpiamater)

CS1a-oneleveldisease Involvesonelevelofthespineorspinalcord:cervical,thoracic,lumbar,orsacral CS1b-oneleveldiseaseandleptomeningealdisease Involvesonelevelofthespineorspinalcord:cervical,thoracic,lumbar,orsacral;with

involvementoftheCSFandleptomeninges(arachnoidandpiamater)

CS2a-twoleveldisease Involvestwolevelsofthespineorspinalcord:cervical,thoracic,lumbar,orsacral CS2b-twoleveldiseaseandleptomeningealdisease Involvestwolevelsofthespineorspinalcord:cervical,thoracic,lumbar,orsacral;

withinvolvementoftheCSFandleptomeninges(arachnoidandpiamater) CS3a-threeleveldisease Involvesthreelevelsofthespineorspinalcord:cervical,thoracic,lumbar,orsacral CS3b-threeleveldiseaseandleptomeningealdisease Involvesthreelevelsofthespineorspinalcord:cervical,thoracic,lumbar,orsacral;

withinvolvementoftheCSFandleptomeninges(arachnoidandpiamater) CS4a-fourleveldisease Involvesfourlevelsofthespineorspinalcord:cervical,thoracic,lumbar,orsacral CS4b-fourleveldiseaseandleptomeningealdisease Involvesfourlevelsofthespineorspinalcord:cervical,thoracic,lumbar,orsacral;

withinvolvementoftheCSFandleptomeninges(arachnoidandpiamater)

Diazatalsuggestedthatallthetumoralrecurrencesoccurin

patientswithkadishstageCtumors[5].Ontheotherhand,many

otherauthorsreportedinliteraturethatthemostfrequenttumoral recurrenceis local; and this highincidance oflocal recurrence isdirectlyrelatedtoinadequatetumorresectionmargins[6].It hasbeendemonstratedinliteraturethatsurgeryaloneseemsto beineffectiveforlocalcontrolofENB,andmanyauthorssuggest postoperativeradiotherapytoreducetheriskoflocalrecurrence of the tumor.In the adjuvant or neo-adjuvant platinum-based chemotherapyinadditiontoradiotherapyhasbeenoftenchoosen forlocalcontrolofrecurrenceordistantmetastasis[13].Although craniofasialresectioncombinedwithradiotherapyisconsidered thegoldstandarttreatmentinmanagementofENBtoday,some seriouscomplicationsarerepresentedaftercraniofasialresection inrecentliterature[7,8].Toavoidfromthesecomplications,recent reports have suggested to treat the ENB with minimally inva-sivesurgery suchas endoscopic approaches which could have someadvantagessuchasshortdurationofsurgery,short hospi-talizationandabetterqualityoflifewithoutanestheticdamage

[6–8]. In present case, beforethe radiotherapy administration,

bilateralendoscopic endonasal resection and lateral rhinotomy approachwhich couldgiveenoughsurgicalcorridortothe sur-geontoremovethetumorfromthenasaldorsumwasprefered andperformedtothepatient.Elevenmonthsaftersurgery,nolocal

recurrenceinnasalcavityandnasaldorsumwasobservedatfollow upexams.

Neckmetastasiscanoccurearlyinthediseaseormanyyears later.Neckdissectionisindicatedonlyinthepresenceofnodes,and electivedisectionappearstobeunnecessary.Theincidenceof dis-tantmetastasis(suchaslung,brain,boneetc.)occurin12%–25%of patients[10].Additionally,metastasistothespinalcordfromENB whichisanuncommoneventpresentsoftenyearsafterinitial diag-nosis.TheincidanceofcervicalmetastasisoftheENBvariesfrom 10%to33%atthetimeofthediagnosis[9].Butthoracalmetastasis oftheENBisvaryrare.Inliteraturenearly30patientswith verte-braldropmetastaseshasbeendocumentedtoday.ENBisalocally aggressivetumorsanditsrecurrenceratioisreported10%to60%. Ontheotherhand,despiteaggressivetreatmentitcommonlycould metastasetothecervicallymphnodesorlungsbeyond1month to10yearsinliterature[11,12].Dropmetastasistospineis clas-sifiedbyCedars-Sinai(SeeTable1)[13]. ˙InadditiontoMoriand Zhangetal.describedcaudaequinametastasesoftheENBtreated withsurgicalresectionandradiationtherapy [12,14].Inpresent case,distantmetastasistotheT10vertebra12monthsafterinitial treatmentanditcouldbediagnosed.

Inconclusion,practitionairsshouldsuspectthedropmetastases intheirpatientswithENBwhosuffersfromthebackand/or radicu-lopathicpain.

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4. Conclusion

Spinal metastasis has beenshown to occurin many

differ-enttumors,butintraduralandcaudaequinametastasesarerare.

Becauseoflocoregionalrecurrencesarecommon,patientsmustbe

followedcarefullyforlocoregionalanddistantmetastasis.

Conflictofinterest

None.

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