Nerve sheath myxoma of the dorsal paravertebral space

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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

Nerve

sheath

myxoma

of

the

dorsal

paravertebral

space

Melih

Malkoc

_,

_Tugrul

_Ormeci

_,

_Mert

_Keskinbora

_,

_Adem

_Yılmaz

_,

_Ozgur

_Korkmaz

_,

Canan

Besleyici

Tanik

a_{DepartmentofOrthopedicsandTraumatology,IstanbulMedipolUniversity,SchoolofMedicine,Turkey} b_{DepartmentofRadiology,IstanbulMedipolUniversity,SchoolofMedicine,Turkey}

c_{DepartmentofNeurosurgery,SisliResearchandEducationHospitalIstanbul,Turkey} d_{DepartmentofPathology,SisliResearchandEducationHospitalIstanbul,Turkey}

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Articlehistory: Received24April2014

Receivedinrevisedform31July2014 Accepted6October2014

Availableonline16October2014

Keywords: Nervesheathtumor Myxoma

Neurothekeoma Paravertebralspace

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INTRODUCTION:Nervesheathmyxomas(NSM)arerarebenignsofttissuetumors.Thedorsal

paraverte-bralplacedNMSdiagnosiscanbedifficult.

PRESENTATIONOFCASE:Thisarticlepresentsclinical,radiologicalfindingsandtreatmentoftheNSMof

thedorsalparavertebralspaceina32-year-oldmanpresentedwitharightshoulderandbackpainfor4

years.

DISCUSSION:NSMisarareandbenigntumorandthatmostoftenoccursintheskinofthehead,neck

orupperlimbsofyoungerpatients.Rarelocationssuchasintracranial,spinalcanal,trunk,lowerlimb

andoralcavitywerealsoreported.TheappropriatetreatmentofNSMissurgicalexcision.Diagnosisis

difficultinanuncommonpresentation.

CONCLUSION:AlthoughthemostpresentedcaseofNMSare dermaltumors,itmay alsobe found

extremelyrarelocations.Weconcludethat,thedefinitivetreatmentofNSMissurgicalexcisionwith

safemarginsevenwhenitispossible.

©2014TheAuthors.PublishedbyElsevierLtd.onbehalfofSurgicalAssociatesLtd.Thisisanopen

accessarticleundertheCCBY-NC-SAlicense(http://creativecommons.org/licenses/by-nc-sa/3.0/).

1. Introduction

Nervesheathmyxomas(NSM)arerarebenignsofttissuetumors thathave apredilictiontohead–neckregionand upperlimbs.1

Although,themajorityofNSMarecommonlydermal,other loca-tionslike mucosal,intracranial and intraspinal have also been described.Wereporta caseofdorsalNSM withmisdiagnoseof nearly4years.Tothebestofourknowledge,itwasthefirstcase ofNSMinvolvingthedorsalparavertebralspace,reportedinthe Englishliteraturetodate.

2. Presentationofcase

A32-year-oldotherwise healthymanpresentedwitharight shoulderandbackpainfor4years.Recently,painstartstocontinue atrestandpatientdescribingdifficultyinfallingasleepandhad difficultytoperformhisdailyactivitiesandworkingschedule.

PositivityofJobetest,dropping sign,Hawkinstest,Yergason testandSpeedtestobservedinthephysicalexaminationofright shoulder.Scapularinstabilitywasdetermined.Thepatientdefines

∗ Correspondingauthorat:DepartmentofOrthopedicandTraumatology,Istanbul MedipolUniversity,SchoolofMedicine,TemAvrupaOtoyoluGoztepeCıkısi,No.1 Bagcilar,34214Istanbul,Turkey.Tel.:+902124607777;fax:+902124607070.

E-mailaddress:[email protected](M.Malkoc).

slighttendernesswithdeeppalpationofspinalprocessbetweenT2 andT5,butnomassidentified.Therewasnomotorandsensorial deficiencydeterminedontheshoulderandarmmusculature.Many differentdiagnosticandtreatmentmodalitieswasperformed ini-tially,likeacupuncture,electromyographyorshoulderandcervical magneticresonanceimaging(MRI).Noneofthesemodalitiescould achievetoshowanabnormalityorreducethepain.AthoracalMRI wasperformedinourhospital.

Magneticresonanceimagingshowedanill-defined,solidtumor locatedintherightparavertebralspacebetweenT2andT4 verte-brae. The lesion extends into thorax with an extrapulmonary fashionatthelevelofT2–3whereitdevelopsdestructiononthe adjacentribandtransverseprocess.Thelesionhadslight heterge-neoushighintensitywhencomparedwithmuscletissueonT1-and brightsignalintensityechoonT2-weightedimages.Enhancement identifiedinthelesionafterintravenouscontrastadministration. Thelesionmeasured15mm×17mmintheaxialplane(Fig.1)and 20mminthecoronalplane(Fig.2).

Inthedifferentialdiagnosisofnervesheathmyxoma, aggres-siveangiomyxoma,myxoidneurofibroma,lowgradefibromyxoid sarcoma,myxoidliposarcoma,lowgrademyxofibrosarcoma, cel-lularmyxoma,juxta-articularmyxoma,nodularfasciitismustbe inmind.Forthisreasonpatientundergoneaclosedbiopsywith administration of local anesthesia and sedation. From a lancet tipsmallincision,aJamshidineedleinsertedbytheguidanceof ComputerizedTomography.Threetissuesamplesweretakenfrom

http://dx.doi.org/10.1016/j.ijscr.2014.10.003

2210-2612/© 2014The Authors.Publishedby Elsevier Ltd.onbehalf of SurgicalAssociates Ltd.This is an openaccessarticle under theCC BY-NC-SA license (http://creativecommons.org/licenses/by-nc-sa/3.0/).

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M.Malkocetal./InternationalJournalofSurgeryCaseReports5(2014)858–860 859

Fig.1. InaxialTSET2MRimage;thereisslightlyheterogeneoushyperintenselesion

isseenatthelevelofT2–T3.

differentpartsofthelesion.Pathologicalinterpretationrevealeda myxomatouslesionwithoutanysignsofmalignancy(Figs.3and4). Completeexcisionofthelesionwasperformedbya longitu-dinalmidlineincisionover thelesionside. Bybluntdissections of therightparavertebral muscles, pseudocapsuleof thelesion wasreached. Although,thecloserelationof themasswiththe nearbyvertebralbodiesandtheribs,noinvasiontothebonytissue wasobserved.Theadjacentpleuralmembranewasintact. Intra-operativefrozensectionwasconfirmedthebenignnatureofthe lesion.Twopiecesof grayish-pinktumortissuewasexcisedby carefulbluntdissections.Accordingtopathologyreporttherewas nomitotic activityand nolipoblasts. The excisedmaterialwas hypocellular,composedofblandcellsandscantbloodvessels.The tumorwasimmunohistochemicallycharacterizedasaNSMgiven itspositiveimmunoreactivityforS-100protein,CD10and epithe-lialmembraneantigen,withnoimmunoreactivitytocytokeratin.

Fig.2.PostcontrastcoronalSPIRTSET1imageshowscontrastenhancedlesion locatedbetweenT2andT4vertebraewithextrapulmonaryfashion.

Fig.3. microscopyofnervesheathmyxoma,redarrowindicatethetumoraltissue, whitearrowindicatestheperipheralnerve.

The patient’s symptoms disappeared immediately after the operation. The post-operative coursewas uneventful. One year afterhisoperation,clinicalfollow-upandMRIshowednoevidence ofarecurrentlesionat3rd,6th,9thandinthelastfollowup1year aftersurgery.Patientreturnedtohisdailyactivitieswithoutpain andrestriction.

3. Discussion

NSMisararetumorthatmostoftenoccursintheskinofthe head,neckorupperlimbsofyoungerpatients.Likeourdorsally paravertebrallocatedcase,somerarelocationssuchasintracranial, spinalcanal,trunk,lowerlimbandoralcavitywerealsoreported. Thereportedagesofthepatientsrangebetween15monthsand 84years.2_{Thepreviouslyreportedcaseshadapeakincidencein}

thefourthdecadeoflife,butareextremelyrareininfancy.3_Ithas

aremarkablepredilectionforthefemalegender.4

Thesebenign lesionswerefirstdescribed in1969byHarkin andReed.Although,thetumorwaslaterre-namedbyGallagerand Helwigasneurothekeoma,recentgenestudiesstronglysupports that, nerve sheath myxomas and neurothekeomas are distinct neoplasms. Schwann-cell differentiation in the ultrastructural observationofNSM cells, suggestsanoriginfromnerve sheath

Fig.4.microscopyofnervesheathmyxoma,redarrowindicatesthetumoraltissue, whitearrowindicatesperipheralnerve.

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860 M.Malkocetal./InternationalJournalofSurgeryCaseReports5(2014)858–860

precursorcells.However,therearestillsometheoriesproposinga proliferationofotherperineuralcells,whichbringscontroversies tothecelloforigin.5_{TherearesomesimilarfeaturesofNSMwith}

otherneuraltissuetumors,likeschwannomaandneurofibroma. That seems the reason why various names such as pacinian neurofibroma, cutaneouis lobular neurofibroma and perineural neurofibromahave beenusedforthislesion.Theseoverlapping featuresareleadingtodifficultiesindiagnosis.

Threevariantsof NSM weredescribed basedon histopatho-logicalevaluation.These subtypesincludethemyxoid type,the cellulartypeandthemixedtype.Theclassicalmyxoidtypeis char-acterizedbylowcellularityandlargeamountsofmyxoidmatrix. ThelesionusuallyborderedanddiffuselypositiveforS-100.Asin ourcase,thestrongpositivityoftumorcellsforS-100,whichis awell-establishedmarkerforSchwanncellsandmyelinsheath, favorsthe Schwann cell origin.The cellulartypes of NSM typ-ically are notwell circumscribed, and themixed type includes variedcellularitywithinfocalmyxoidregions.6,7_{Thedifferential}

diagnosesinclude schwannoma (alsothemaligntype), neurofi-broma,neurilemmoma,leiomyoma,intramuscularmyxoma and low-gradesarcoma.

TheappropriatetreatmentofNSMissurgicalexcision.Inour case,thecloserelationofthetumoralmasswithpleuraand adja-centvertebra-ribjunctionmakesthetotalexcisiondifficulttoget withsafe margins. Afteran intra-operative frozen section that revealedthebenignnatureofthelesion,amarginalexcisionwas performed.Anintra-operativeconsultationfromthoracicsurgery wasalsotaken,inordernottopenetratethepleuralmembrane. Althoughamarginal excisionwasperformedinourpatient,no recurrenceobservedinthepatientsfirstyearfollow-up.Usually, NSMsupposed to havea low recurrence rateafterlocal recur-rence,butsomeauthorsreporteda recurrencerateinabout7% ofpatients.8,9

4. Conclusion

Althoughthemajorityofreportedcasesaredermaltumors,NSM mayalsobelocatedinthe extremelyrare locations.Weaimed toincrease theawareness aboutthis disease,in orderto avoid delays in diagnosis.We concludethat, thedefinitivetreatment of NSM is surgical excision withsafe marginseven when it is possible. Conflictofinterest None. Funding None. Ethicalapproval

Writteninformedconsentwasobtainedfromthepatientfor publicationofthiscasereportandaccompanyingimages.

Authorcontributions

MelihMalkoccontributedtostudydesign.TugrulOrmeci con-tributedtodatacollecting.MertKeskinboracontributedtowriting. AdemYılmazcontributedtodatacollectingandanalysis.Ozgur Korkmazcontributedtofigurepreparation.CananBesleyiciTanik contributedtopathologicspecimenanalysis.

References

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2.PapadopoulosEJ,CohenPR,HebertAA.Neurothekeoma: reportofacasein aninfantandreviewoftheliterature.JAmAcad Dermatol2004;50(January (1)):129–34.

3.FetschJF,LaskinWB,MiettinenM.Nervesheathmyxoma:aclinicopathologicand immunohistochemicalanalysisof57morphologicallydistinctive,S-100 protein-andGFAP-positive,myxoidperipheralnervesheathtumorswitha predilec-tionfor theextremities andahigh localrecurrencerate.AmJSurgPathol 2005;29(December(12)):1615–24.

4.O’RourkeH,MeyersSP,KatzmanPJ.Neurothekeomaintheupperextremity: magneticresonanceimagingandcomputedtomographyfindings.JComputAssist Tomogr2005;29(November–December(6)):847–50.

5.GallagerRL,HelwigEB.Neurothekeoma–abenigncutaneoustumorofneural origin.AmJClinPathol1980;74(December(6)):759–64.

6.LeeD,SuhYL,HanJ,KimES.Spinalnervesheathmyxoma(neurothekeoma).Pathol Int2006;56(March(3)):144–9.

7.AkhtarK,ZaheerS,RayPS,SherwaniRK.Myxoidneurothekeoma:araresofttissue tumorofhandinamaletoddler.NigerJSurg2013Jan;19(1):32–4.

8.HornickJL,FletcherCD.Cellularneurothekeoma:detailedcharacterizationina seriesof133cases.AmJSurgPathol2007;31:329–40.

9.WollinaU,HanselG,SchönlebeJ,HaroskeG.Myxoidneurothekeoma–apainful caseinalesscommonlocation.DermatolOnlineJ2009;15(April(4)):3.

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