case report successful treatment of recurrent chordoma and bilateral pulmonary metastases following an 11 year disease free period

c o m Case report: Successful treatment of recurrent chordoma and bilateral pulmonary metastases following an 11-year disease-free period Cherie P.. 1.Axial CT slices with arrows indicat

International Journal of Surgery Case Reports xxx (2014) xxx–xxx

International Journal of Surgery Case Reports

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

Case report: Successful treatment of recurrent chordoma and bilateral

pulmonary metastases following an 11-year disease-free period

Cherie P Erkmena,∗, Richard J Barthb, Vignesh Ramana

Q1

a Division of Thoracic Surgery, Department of Surgery, Dartmouth-Hitchcock Medical Center, One Medical Center Drive, Lebanon, NH 03756, United States

b Section of Surgical Oncology, Department of Surgery, Dartmouth-Hitchcock Medical Center, One Medical Center Drive, Lebanon, NH 03756, United States

a r t i c l e i n f o

Article history:

Received 8 November 2013

Received in revised form 1 February 2014

Accepted 7 February 2014

Available online xxx

Keywords:

Chordoma

Recurrence

Metastasis

a b s t r a c t

INTRODUCTION:Chordomasarerarebutaggressivetumorsduetolocalrecurrenceandraredistant metas-tases.Theyoriginatecommonlyinthesphenooccipitalandsacrococcygealregions,andmetastasizeto thelungs,bone,skin,liver,andlymphnodes.Theyoccurmorefrequentlyinmenandpeopleoverthe ageof40

PRESENTATIONOFCASE:A28year-oldfemalepresentedwithsacrococcygealchordomaforwhichshe receivedwidelocalexcisionandadjuvantradiationtherapy.Sheenjoyedanunusualdisease-free sur-vivalfor11yearsuntilaroutinesurveillancescanofthepelvisidentifiedlocalrecurrence.Furtherwork

uprevealedbilateralpulmonarymetastases.Sheunderwentlocalexcisionoftherecurrenttumorand video-assistedthoracoscopic(VATS)wedgeresectionofpulmonarymetastases.Shealsoreceived adju-vantradiationtherapytotherecurrentresectionbed.Twoyearslater,sheremainsfreeofdiseaseand symptoms

DISCUSSION:Chordomasarecommonlyinsensitivetochemotherapyandradiation,makingsurgerythe mostsuccessfultherapeuticmodality.However,therearefewguidelinesonthesurveillanceand treat-mentofrecurrentchordoma.Wereportsuccesswithaggressivesurgicalresectionofrecurrenceand metastasisaswellasadjuvantradiationtherapy

CONCLUSION:Theprolongedsurvivalofourpatientunderscorestheimportanceof(1)aggressivesurgical resectionofchordoma,whetherprimary,recurrent,ormetastatic,withadjuvantradiationtherapy,(2) minimizationofsurgicalseedingoftumor,and(3)diligentcancersurveillance

©2014TheAuthors.PublishedbyElsevierLtd.onbehalfofSurgicalAssociatesLtd.Thisisanopen accessarticleundertheCCBY-NC-NDlicense(http://creativecommons.org/licenses/by-nc-nd/3.0/)

Chordomas are locally invasive, aggressive tumors of

noto-chordalremnants.Chordomashaveageneralincidenceof0.08per

100,000peopleandconstitute1–4%ofallbonecancers.1Theyare

especiallyrareinpeopleyoungerthan40andmorefrequentin

menthanwomen.2Themediansurvivalis6.29yearswitha10year

survivalof39.9%.2Mostprimarytumorsoriginateinthecranium

(32.0%),spine(32.8%),orsacrum(29.2%).2 Extra-axialmetastasis

occursin3–48%ofpatients.3–5Completesurgicalresectionisthe

mainmodality oftreatment.Chordomas arefrequently

insensi-tivetochemotherapyandradiationtherapy Localrecurrenceis

themostimportantindicatorofpoorprognosis.6Metastasis,

com-monlyoccurringinthelungs,liver,bone,lymphnodes,andskin,

isoftenassociatedwithlocalrecurrenceoftheprimarytumor.5

Here,wedescribetheclinicalcourseandexaminetheoutcomes

ofourmanagementofa39-year-oldwomanwhopresentedwith

∗ Corresponding author Tel.: +1 603 650 8537; fax: +1 603 650 6346.

E-mail address: Cherie.P.Erkmen@Hitchcock.org (C.P Erkmen).

multifocal metastases to thelungs and local recurrencein the glutealregion11yearsafterresectionofasacralchordoma

2.1 Primarytreatment:aggressiveresectionwithadjuvant radiation

A 28-year-oldwoman presentedwithright buttock discom-fortincreasing over fiveyears Physicalexamination revealeda firm,immobilemass,posteriortotherectum.MRIdemonstrated

a20cmmassoriginatingfromthesacrumatthelevelofS5and extendingintothegluteusmuscles.ACT-guidedbiopsyofthemass demonstratedchordoma.Thepatientunderwentenblocresection

ofthetumorandrightverticalrectusabdominusmyocutaneous flapforperinealreconstruction.A6-weekcourseofadjuvant exter-nalbeamradiationtherapy (6000cGy)wasadministered Apart fromoccasionalfecalincontinence,thepatientremainedotherwise asymptomaticfor11yearswithnoevidenceoflocalrecurrenceor metastasis.Duringthisperiod,sheunderwentyearlysurveillance abdomenandpelviscontrastenhancedCTscans

http://dx.doi.org/10.1016/j.ijscr.2014.02.005

2210-2612/© 2014 The Authors Published by Elsevier Ltd on behalf of Surgical Associates Ltd This is an open access article under the CC BY-NC-ND license

(http://creativecommons.org/licenses/by-nc-nd/3.0/).

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Fig 1.Axial CT slices with arrows indicating (a) local recurrence of chordoma posterior to right gluteus maximus, (b) mass eventually identified as a lipoma in the left sartorius muscle, (c) metastatic chordoma nodule in left lower lobe of the lung, and (d) metastatic chordoma nodule in right middle lobe of the lung.

revealedpulmonarynodules(Fig.1candd).AdedicatedCTscanof

thechestsubsequentlyidentifiedfourdiscretepulmonarylesions:

19mmnoduleintherightmiddlelobe,3mmnoduleinthe

supe-riorsegmentoftherightlowerlobe,10mmnoduleintheleftlower

lobe,anda10mmnoduleintheleftupperlobe.Onlytheright

mid-dlelobelesionwashypermetabolicbyPETscan.HeadMRIshowed

noevidenceofintracranialmetastasis.CTguidedbiopsyoftheright

middlelobenoduleshowedmetastaticchordomaconsistentwith

theoriginalsacralchordoma.Wedidnotbiopsythesuspectedsite

oflocalrecurrence

Given the appearance of locally recurrent disease with

oligometastaticdisease,weendeavoredtoresectallsuspectedfoci

ofdiseasewithcurativeintent.Inanefforttominimizethe

num-berofoperationsduetothepatient’slimitedfinancialmeanswhile

avoidingbilaterallungsurgeryatthesamesetting,weplannedtwo

surgeries.Thepatientfirstunderwentvideoassistedthoracoscopic

surgery(VATS)removalanddiagnosticbiopsyofthetwoleft-sided

pulmonarynodules.Bothleftlungnodulesweremetastatic

chor-domaresectedwithnegativemargins.Amonthlater,thepatient

hadwidelocalexcisionofrightbuttockmass,rightsartoriusmass,

andVATSresectionoftworight-sidedpulmonarynodules.Alllung

specimensremoved viaa retrievalbag (ENDOPOUCHSpecimen

RetrievalBagSystem,Ethicon,Cincinnati,OH).Therightbuttock

masswasdedifferentiatedchordomawithahigh-gradespindlecell

sarcomatouselementandnegativemargins(Fig.2).Thesartorius

masswasanintramuscularlipoma.Therightmiddlelobelesion

waschordomawithnegativemargins;therightlowerlobelesion

wasanintrapulmonarylymphnode.Thefinaldiagnosiswaslocal

recurrenceanddedifferentiationofsacralchordomawithbilateral

pulmonarymetastases demonstrating originalchordoma histol-ogy.Aftersurgery,thepatientreceived4600cGyofradiationin

200cGydosestotheregionoftherecurrentrightbuttock chor-doma

2.3 Long-termfollow-up Sheisfreeofdiseaseandsymptomstwoyearsafterher recur-rencewithmetastasesand13yearsafterherinitialdiagnosisof chordoma She hascontinued to undergo surveillance contrast enhanced CTscans ofher chest,abdomen,and pelviseverysix months

3.1 Pathologicalchangeinlocalrecurrence Dedifferentiationofchordomahasbeenreportedrarelyinlocal recurrence,andhasbeenassociatedwithpoorprognosis.7–9Itis unclearifsarcomatoustransformationisdueto(1)spontaneous changeoftheoriginalchordoma,(2)irradiation-inducedchange,or (3)polyclonalconvergenceoftwodifferentcancers.9Ourpatient

isthefirstknownreportofdedifferentiatedchordomarecurrence with differentiated chordoma pulmonary metastases The lung metastases couldhave occurredearly,beforetransformation of theinitialchordomaandtakenanoccult,insidiouscourse,orthey couldhaveevolvedfromdifferentiatedchordomacellsina poly-clonaltumorrecurrence.Theliteraturesupportsthelatterscenario

aslocalrecurrencetypicallyprecedesmetastasis.18

3.2 Surgery Aggressiveenblocsurgicaltreatmenthasbeenassociatedwith decreased localrecurrencerates,decreased incidenceof metas-tasis,andoverallimprovedsurvival.3,10Sincechordomastendto

belocallydestructivetumors,enblocresectionmayrequiretissue

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C.P Erkmen et al / International Journal of Surgery Case Reports xxx (2014) xxx–xxx 3

Fig 2.Pathology H&E stains of gluteal mass showing (a) chordoma cells with characteristic physaliferous cytoplasm and myxoid stroma (20×), (b) lobulated growth pattern

of the tumor (4×), (c) zonal necrosis in the area of the tumor (4×), and (d) tumor invading the bone (4×).

tumorwithnegativetissuemarginsandreconstructionofher

per-ineumledtoalongdiseasefreesurvivalafterinitialtreatment.We

thereforeadvocateaggressiveinitialsurgicalresection

Localrecurrencecanoriginatefromseedingofthetumorduring

biopsy,resectionoftheprimarytumor,orreconstruction.12,13To

minimizethelocalrecurrence,werecommend(1)wideresection

margins,(2)minimalhandlingofthetumor,and(3)new

instru-ments,andglovesbeusedforreconstructionandclosure.13With

pulmonarymetastasis,weadvocateaVATSwedgeresectionwith

widemarginsandspecimenremovalviaaretrievalbagtoprevent

tumorcontaminationofportsites

3.3 Radiationtherapy

Theuseofradiotherapytotreatprimaryandsecondary

chor-doma is controversial,mitigated by theintolerance ofadjacent

spinalcordand brainstemtohighradiationdoses.14 Highdose

radiotherapywithsurgeryhasbeenproventofavorablyaffect

dis-easefreeinterval.15 Conventionalradiationtherapy at40–60Gy

hasresultedin5-yearlocalcontrolof10–40%.14,16Ourpatientwas

treatedwithadjuvantradiationtherapyforsixmonthsfollowing

initialresectionandalsoreceivedadjuvantradiationtothesiteof

localrecurrence.Whiletheefficacyofadjuvantradiationtherapyin

thesettingofrecurrenceisunknown,17adjuvantradiationtherapy

maybeacontributortohercontinuedlong-termsurvival

3.4 Follow-up

Therearepoorevidence-basedrecommendationsonchordoma

patientfollow-up After initialtreatment, patientsare followed

withfrequentofficevisitsintheimmediatepost-operativeperiod

andthenyearlysurveillanceabdomenandpelvisCTscans

Clini-calsuspicionforlocalrecurrenceshouldbehigherinpatientswho

underwentincompleteexcisionof theiroriginal chordoma.Any

newnodules,fistulas,drainingsinuses,orpainanddiscomfortin

anareaevenremotefromthepreviousexcisionwarrant investi-gationforpossiblerecurrence.Localrecurrencetypicallyprecedes metastasis.18Therefore,werecommendthat patientswithlocal recurrenceshouldundergoanevaluationformetastasiswithfull bodyPET/CTscanandheadMRI.Theroleofroutinesurveillancefor distantmetastasisisunknownatthistime

3.5 Treatmentofrecurrentandmetastaticdisease Evenwithrecurrenceandmetastasis,long-termsurvivalcanbe achievedwithsuccessfulsurgicalresectionofallappreciable dis-ease,asseenwithourpatient.Aggressiveenblocresectionofthe recurrenttumorwithseedingprecautionsisagainpreferred.There arenoreports, letaloneconsensus,aboutthetreatmentof pul-monarymetastasesinchordoma.Wethereforeextrapolatedour experiencewith otherpulmonary metastasis,electing forVATS wedgeresectionsofeachofthepulmonarynoduleswithnegative margins.19,20 For metastatic chordoma to the lung, we recom-mendVATSresectionif(1)theprimaryor recurrenttumorcan

becompletelyresected(2)therearenoothermetastases,and(3) thelungmetastasescanberesectedwithamplemarginswithout compromisingthepatient’squalityoflife.Evenmultiple,bilateral pulmonarymetastasescanbeaggressivelytreatedwithlong-term success

Thetreatmentofchordomademandsbothaggressiveresection and cautious attention tominimizing surgicaltumor implanta-tion.Adjuvanttherapy iscontroversialbutknowntocontribute

tolong-termdiseasecontrolandsurvival Postoperativepatient follow-upshouldcontinuelong-term;signsorsymptoms consis-tentwithpossiblelocalrecurrenceormetastasisshouldprompt

athoroughwork-up.Intheabsenceofsymptoms,surveillanceCT scanscandetectpotentialtreatablerecurrence.Recurrentdisease

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Funding

None

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