位于頸3的上頸椎脊索瘤

Author：LiangJiang

作者：姜亮

OrthopaedicDepartment,PekingUniversityThirdHospital,

單位：北醫(yī)三院現(xiàn)在是1頁(yè)\一共有28頁(yè)\編輯于星期三Chordomaisararelow-grademalignantneoplasmderivedfromtheremnantsoftheembryonicnotochord.Thislocallyinvasiveneoplasmissubjecttorecurrenceaftertreatment.Themediansurvivaltimeisestimatedtobe6.3years.

定義：脊索瘤是一種少見(jiàn)的起源于胚胎脊索殘留組織的低分化惡性腫瘤。

特點(diǎn)：容易復(fù)發(fā)。

統(tǒng)計(jì)學(xué)：中位生存期是6.3年。

Abstract現(xiàn)在是2頁(yè)\一共有28頁(yè)\編輯于星期三Varioustreatmentapproacheshavebeenattempted,includingradicalexcision,radiotherapyandchemotherapy.Treatmentoutcomeissignificantlyinfluencedbythesizeandsiteofthechordoma.Recently,Imatinib,amolecular-targetedagent,hasbeenshowntohaveantitumoractivityinchordoma.Protonradiotherapy,stereotacticradiotherapyandintensity-modulatedradiotherapyhavealsobeenused.Surgicaltreatmentisstilltheprimarychoiceforchordoma.Ithasbecomemoreaggressiveinrecentyears,evolvingfromintralesionalorpartialexcisiontoenblocresection.However,uppercervical

localizationsmakesuchenblocresectioninmostcasesnotpossible.

治療進(jìn)展：傳統(tǒng)治療包括根治性切除、放療和化療等?！癐matinib”的分子靶向治療藥物顯示對(duì)于脊索瘤有治療效果。光子放療，stereotactic放療以及調(diào)強(qiáng)放療亦應(yīng)用于脊索瘤的治療。外科治療仍舊是脊索瘤的首選治療方法。近一些年脊索瘤的外科治療從瘤內(nèi)刮除發(fā)展至部分切除直至全脊椎大塊切除。然而，上頸椎獨(dú)特的解剖位置使大塊全脊椎切除治療脊索瘤很難實(shí)現(xiàn)。

Abstract現(xiàn)在是3頁(yè)\一共有28頁(yè)\編輯于星期三

Wepresentanddiscussthetherapeuticchallengesofayoungfemalewithlargeretropharyngealchordomawhopresentedtoourinstitutionafterconventionalphotonbeamradiotherapy.ThisC2/3tumorwasclassifiedIBaccordingtotheEnnekingclassification.ItdistributedtolayersA–Dandsectors1–6accordingtotheWeinsteinBorianiBiaginiClassification.Theleftvertebralartery(VA)wasencapsulatedanddisplaced.Onestageintralesionalextracapsulartumorexcisionandreconstructionwasachievedbycombinedbilateralhighanteriorcervicalapproachesandposteriorapproach.Norecurrenceormetastasiswasobserved3yearsaftertheoperation.Shereturnedtoherpreviousoccupationasofficeworker.

本文內(nèi)容：我們?cè)谶@里報(bào)道一位經(jīng)過(guò)放療的年輕的咽后脊索瘤女性患者。根據(jù)Enneking分期為IB期，根據(jù)WBB分期則為1-6區(qū)A-D層，左側(cè)椎動(dòng)脈被包繞并且移位。一期采用上頸椎聯(lián)合雙前外側(cè)切口以及后側(cè)入路對(duì)該例患者成功實(shí)施了囊外切除術(shù)。術(shù)后3年隨訪患者無(wú)局部復(fù)發(fā)以及轉(zhuǎn)移。

Abstract現(xiàn)在是4頁(yè)\一共有28頁(yè)\編輯于星期三

Keywords

Keywords

ChordomaAtlantoaxialspineSurgery

關(guān)鍵詞脊索瘤寰樞椎外科手術(shù)現(xiàn)在是5頁(yè)\一共有28頁(yè)\編輯于星期三

A26-year-oldfemalepresentedtoourinstitutionafteratransoralbiopsyofalargeretropharyngealtumor,whichwasdiagnosedasachordoma.Shehaddysphagiaandneckpain9monthsago.Theretropharyngealmasswasnoticedbyotolaryngologist.Aftertransoralbiopsy,shehadconventionalphotonbeamradiotherapywith40Gyandthenhersymptomstotallydisappeared.Sincetherewasnoreductionoftumorvolume6monthsafterradiotherapy,shewasreferredtoourdepartment.Shehadnoothersignificantmedicalhistoryandshedidnotexhibitanyneurologicaldeficit.

年齡：26歲

性別：女性

病史：患者9月前出現(xiàn)頸痛并吞咽困難，首先是由耳鼻喉科醫(yī)生發(fā)現(xiàn)咽后腫物。在經(jīng)口活檢確診后患者接受了40個(gè)Gy的放療，癥狀完全消失。由于放療后6月瘤體未見(jiàn)縮小，患者遂來(lái)就診?；颊呒韧w健，無(wú)神經(jīng)損傷癥狀。

臨床資料現(xiàn)在是6頁(yè)\一共有28頁(yè)\編輯于星期三Fig.1張口位像可以看到頸2椎體左側(cè)邊界不清的溶骨性改變。CT平掃可以看到頸2-3蟲(chóng)蝕樣骨質(zhì)破壞。矢狀位MRI可以看到腫瘤從齒狀突基底延伸至頸3椎體。在軸位像上腫瘤壓迫硬膜囊以及左側(cè)頸內(nèi)動(dòng)脈。左椎動(dòng)脈被包繞并移位。脊髓被擠向右側(cè)。根據(jù)WBB分區(qū)病變位于A-D層和1-6區(qū)，根據(jù)Enneking分期為IB期。現(xiàn)在是7頁(yè)\一共有28頁(yè)\編輯于星期三Fig.2Layers(A–D)andQuadrantsinvolvementaccordingtoWeinsteinBorianiClassification(normalcervicalvertebrarepresented)

現(xiàn)在是8頁(yè)\一共有28頁(yè)\編輯于星期三Chordomaisarelativelyrarelow-grademalignantneoplasmderivingfromremnantsofthenotochord,constitutingbetween1and4%ofmalignantbonetumors.Itpredominantlyarisesfromtheaxialskeleton,whileextra-axialchordomahasalsobeenreported.Itsincidencehasbeenestimatedtobeoneperonemillioninhabitantsperyear.Itcanaffectpatientsfromallageswithapredilectionformenwitharatioof1.6–1(10).Thefrequentlyreportedanatomicdistributionofchordoma,sacral*50%,spheno-occipital*35%,spinal*15%,wasbasedprimarilyon262patientsfromtheMayoClinicexperience.Basedonmorerecent400casesfromtheUnitedStates[10],32%ofcaseswerepresentedatcranialsites,33%spinaland29%sacral.Medianageisaround60years,butskull-basepresentationsaffectayoungerage,andmayevenoccurinchildrenandadolescents.

脊索瘤是少見(jiàn)的起源于胚胎殘留脊索組織的低分化惡性腫瘤，占惡性骨腫瘤的1-4%，主要起源于中軸骨骼系統(tǒng)，其他部位也見(jiàn)報(bào)道。統(tǒng)計(jì)學(xué)顯示其每年發(fā)病率為百萬(wàn)分之一。各個(gè)年齡段均可發(fā)病，男性多于女性，男女比率為1.6：1。美國(guó)紐約的Mayo醫(yī)學(xué)中心統(tǒng)計(jì)262例脊索瘤按照解剖部位分類，蝶枕部為35%，骶骨50%。其他脊柱階段15%。美國(guó)最近的400例病例統(tǒng)計(jì)，32%位于顱部，33%脊柱，29%位于骶骨。平均發(fā)病年齡為60歲，顱底部脊索瘤發(fā)病年齡較輕，甚至見(jiàn)于兒童和青少年。

診斷和流行病學(xué)現(xiàn)在是9頁(yè)\一共有28頁(yè)\編輯于星期三Chordomaischaracteristicallyslow-growing,locallyinvasiveneoplasm.Inmostcases,theEnnekingstageisIB—lowgrademalignant,extracompartmental.Additionally,theycanbedifficulttodistinguishradiographicallyandpathologicallyfromchondrosarcoma.Sometimes,chordomahasdumbbellshapewhichcouldbediagnosedasneuromas.Spinechordomashavealsobeendiagnosedastuberculosisandhemangioma.ThemainstaytoachieveappropriatediagnosisisabiopsyperformedbytrocarunderCTscancontrolwiththebiopsytrackthatcanbeexcisedduringthesurgicalexcision.

脊索瘤具有緩慢生長(zhǎng)、局部侵襲的特點(diǎn)。大部分病例為IB期-低度惡性、間室外。依靠影像學(xué)和病理學(xué)資料仍舊難以將其與軟骨肉瘤鑒別。有時(shí)具有扇貝樣表現(xiàn)被誤診為神經(jīng)瘤。脊柱脊索瘤也有誤診為結(jié)核和血管瘤的報(bào)道。獲得正確診斷的主要依據(jù)是在CT引導(dǎo)下用套管針行穿刺活檢，穿刺路徑可以在之后的外科手術(shù)中切除。

診斷和流行病學(xué)現(xiàn)在是10頁(yè)\一共有28頁(yè)\編輯于星期三Metastasesarerareandoccurtothelungortothespine,exceptionallytheycanoccurinothersitesofthemusculoskeletalsystemorevenintheskin,brain,andotherorgans.Thesurvivalrateappearstobeaffectedmorebylocaltumorprogressionthanbymetastasis.Thelifeexpectancyfromdiagnosisis6monthsto2yearsintheuntreatedskullbasechordomapatient.Mediansurvivalwas6.3yearsintheSurveillance,Epidemiology,andEndResults(SEER)programoftheNationalCancerInstitute,lookingata22yearsdatabaserecord.IntheUnitedStatesthe5-and10-yearrelativesurvivalrateswere67.6and39.9%,respectively.Therewerenosignificantdifferencesinsurvivalalthoughmediansurvivalwasslightlylowerinspinalchordomas(5.9years)comparedtocranial(6.9years)andsacral(6.5years)presentations.

脊索瘤少見(jiàn)轉(zhuǎn)移，一般轉(zhuǎn)移至肺臟或者脊柱，偶爾也可轉(zhuǎn)移至肌骨系統(tǒng)其它部位或者皮膚、顱內(nèi)等臟器。生存率多與原發(fā)灶關(guān)系密切而與轉(zhuǎn)移灶則關(guān)系甚微。未經(jīng)治療的顱底脊索瘤從診斷開(kāi)始預(yù)計(jì)生存期為6月至2年。美國(guó)國(guó)家腫瘤研究中心22年數(shù)據(jù)統(tǒng)計(jì)顯示其脊索瘤中位生存期為6.3年。在美國(guó)其5年和10年生存率分別是67.6%和39.9%。盡管脊柱脊索瘤的中位生存率稍低，但是與顱底和骶骨相比并無(wú)明顯統(tǒng)計(jì)學(xué)差異。

診斷和流行病學(xué)現(xiàn)在是11頁(yè)\一共有28頁(yè)\編輯于星期三Becauseoftheirlocation,behavior,andtheconfusingdiagnosticpicture,chordomascanbedifficulttomanage.Treatmentoutcomeissignificantlyinfluencedbythesizeandsiteofthechordoma.Ingeneral,cervicalchordomasaresouncommonthatarticleshavereportedlong-termfollow-upevaluationofsuchtumorsonacase-by-casebasisonly.Atlantoaxialchordomaisextremelydifficulttotreatmainlybecauseofitslocationclosetovitalstructures.SincethereisalargeduralspaceattheC1–C2location,thetumorusuallygetsquitelargebeforethepatienthassomeclinicalmanifestation.Compressionofneuraltissuesandinvasionofthebrainstemorspinalcanalmayresultinseriousneurologiccomplications.Itoftenencapsulatesand/ordisplacesthevertebralarteryandnerveroots.

由于脊索瘤發(fā)病部位、生物學(xué)行為以及難以診斷，治療較難。療效明顯與腫瘤的大小、部位相關(guān)。一般而言，頸椎的脊索瘤非常少見(jiàn)，長(zhǎng)期隨訪結(jié)果都是建立在個(gè)案報(bào)道基礎(chǔ)之上。

由于寰樞椎臨近較多重要的解剖結(jié)構(gòu)，治療尤為困難。由于C1-2椎管寬闊，患者往往在腫瘤相當(dāng)大時(shí)方出現(xiàn)臨床癥狀。壓迫神經(jīng)組織以及侵襲腦干或者椎管可以導(dǎo)致嚴(yán)重的神經(jīng)系統(tǒng)并發(fā)癥。腫瘤經(jīng)常侵襲椎動(dòng)脈和神經(jīng)根并使其移位。

診斷和流行病學(xué)現(xiàn)在是12頁(yè)\一共有28頁(yè)\編輯于星期三Varioustreatmentapproacheshavebeenattempted,includingisolatedradiotherapy,combinedradiationandsurgicalexcision,andsurgicalexcisionalone.Surgeryremainsthetreatmentmainstayofchordoma.Excellentteamworkisneededforaggressiveandsuccessfulsurgicaltreatmentinmostcases.Thesefactorspleadforearlyreferraltoatertiarycarecenterwithadvancedsurgicalcapabilitiesandavailabilityofnewermodalitiesofradiationtherapy.

對(duì)脊索瘤已經(jīng)采取包括適形放療、外科手術(shù)治療和二者結(jié)合的治療法方法。外科治療仍舊是脊索瘤的首選方法。成功的外科手術(shù)需要優(yōu)秀的醫(yī)生團(tuán)隊(duì)，并且需要早期在具有專業(yè)外科技術(shù)和放療設(shè)備的中心進(jìn)行治療。治療方法和文獻(xiàn)依據(jù)現(xiàn)在是13頁(yè)\一共有28頁(yè)\編輯于星期三Chemotherapyhaslongbeenknowntobeinactiveinchordoma.Italonemightbeanoptionforsupportivecareoncethediseaseextendsandprogresses.Cisplatincouldbeusedlocallyaftertumorresection,ifmarginaltumorcontaminationissuspected.Recently,Imatinib,aproteintyrosinekinaseinhibitorandamolecular-targetedagent,hasbeenshowntohaveantitumoractivityinchordoma[9].Themechanismispoorlyunderstood,althoughblockadeofplatelet-derivedgrowthfactor(PDGF)signalinghasbeenproposedasthecauseoftumorcontrol.

脊索瘤對(duì)化療不敏感。但是當(dāng)疾病進(jìn)展時(shí)化療又成為唯一的選擇。在腫瘤切除后如果懷疑邊界被腫瘤污染，可以在局部應(yīng)用順鉑。最近有一種稱作Imatinib的分子靶向治療藥物顯示對(duì)于脊索瘤有治療效果。盡管血小板源性生長(zhǎng)因子信號(hào)傳遞阻止被懷疑為抑制腫瘤的機(jī)制，但其機(jī)理仍不清楚。治療方法現(xiàn)在是14頁(yè)\一共有28頁(yè)\編輯于星期三Chordomasweretraditionallyrecognizedasradioresistant.Thetolerancedoseofthespinalcordislowerthanthecurativedose,whichshouldatleastbeinthe70Gyrange[3].Butradiationisvaluable,especiallyforincompleteexcision.Inconventionalphotonradiotherapyadoseoflessthan45–50Gyiswellwithintheradiationtolerance,givenlessthana5%probabilityofmyelopathywithin5years.However,withtheadventofnewradiationmodalities(i.e.theprotonbeam,chargedcarbonions,stereotacticradiotherapyandintensity-modulatedradiotherapy),itispossibletodeliverhighdosesofradiationwhileprotectingvitalstructures.Adjuvantradiationisnowwarrantedandhasofferedimprovedclinicaloutcomes.Thereisextensiveexperienceinthemanagementofchordomaswithprotonbeamtherapy,andfavorableresultshavebeendemonstrated[6].Fortheradiationoncologistitiseasiertoidentifythetumormarginbeforethesurgery,butsuchpreoperativeradiotherapymightleadtohigheroperativeandpostoperativecomplicationrates.Postoperativeradiationismorecommoninclinicalpractice,butthetumormarginismoredifficulttoidentifyandthemetalimplantsmightdisturbtheradiationefficacy.Radiation

portsalsoneedtobeconfiguredtotreatthesurgicalrouteandthetumorbedinordertoreducetheriskofsurgicalseeding.Thecost-effectivenessratioforcarbonradiotherapyis2,539€per1%increaseinsurvival,or7,692€peradditionallifeyear[7].

Mostauthorssuggestthatmaximaltumorexcisionsurgeryfollowedbyhigh-doseradiotherapyshouldbethestandardofcareforthesepatients,whileBorianihavedemonstratedthatmargin-freeen-blocresectionaloneisenough.

放療現(xiàn)在是15頁(yè)\一共有28頁(yè)\編輯于星期三既往認(rèn)為脊索瘤對(duì)放療不敏感。放療治療劑量至少需要70Gy，但是脊髓的承受劑量低于此水平。但是放療對(duì)于未能完全切除的脊索瘤是很有必要的。普通放療中患者的承受劑量范圍是小于40-50Gy，并且5年內(nèi)發(fā)生放射性脊髓病的幾率小于5%。然而新放療技術(shù)的應(yīng)用，可以在給予大劑量放療射線的同時(shí)保護(hù)重要組織。輔助放療目前已經(jīng)被認(rèn)可，取得較好的臨床療效。采用光子射線治療脊索瘤目前已經(jīng)取得較好的療效，并積累了豐富的經(jīng)驗(yàn)。對(duì)腫瘤放療專家而言，在術(shù)前比術(shù)后更容易確認(rèn)腫瘤邊界。但是術(shù)前放療增加了手術(shù)以及術(shù)后并發(fā)癥的發(fā)生率。術(shù)后放療在臨床上應(yīng)用更為普遍，但是腫瘤邊界更難確定，并且內(nèi)植物會(huì)影響放療效果。要調(diào)整放療設(shè)備來(lái)照射手術(shù)徑路以及腫瘤邊界以降低外科腫瘤種植的風(fēng)險(xiǎn)。費(fèi)用量效比率為生存率增加1%的代價(jià)為2539元，或者每延長(zhǎng)生存期1年代價(jià)為7692元。

大部分的醫(yī)生認(rèn)為最大范圍的腫瘤切除和最大量的放療應(yīng)該作為治療的標(biāo)準(zhǔn)。但是Boriani認(rèn)為沿邊界行全椎體切除已經(jīng)足夠。

放療現(xiàn)在是16頁(yè)\一共有28頁(yè)\編輯于星期三Chordomasarecharacterizedbyfrequentrecurrencesafterincompletesurgicalresection,usuallywithin2–3years.Boriani,statesthat‘‘Prognosisisheavilyaffectedbytheappropriatenessofthefirstsurgicaltreatment;oncerecurred,furthertreatmentsarealwaysunsuccessfulatmidterm.’’Othersmaypreferincompletesurgicalexcisiontopreserveinitiallytheneurologicfunction,despitetheriskofincreasedrecurrencethatwillleadto

furtherpalliativesurgery.

Surgicaltreatmenthasbecomemoreaggressiveinrecentyears,evolvingfromintralesionalorpartialexcisiontoenblocresection.In1997,Weinstein,Borianietal.describedtheirClassificationsystemforsurgicalstagingforspinetumors.Suchsystemisbasedondividingthevertebrain12quadrantsanddifferentlayersoftumorinvolvementfromAtoD.Tomitadescribedtotalenblocspondyloectomy(TES)usingathreadwireT-sawformalignantvertebraltumorsinthethoracolumbarspine.Toachieveawideresectionmargin,Borianiandhiscolleaguesevenremovedthedurainfiltratedbytumorandreconstructedtheduradefectwithduralpatch.

外科治療現(xiàn)在是17頁(yè)\一共有28頁(yè)\編輯于星期三脊索瘤的特點(diǎn)是外科不全切除后較高的復(fù)發(fā)率，一般是在2-3年內(nèi)。Boriani認(rèn)為預(yù)后受初次手術(shù)正確與否影響極大，一旦復(fù)發(fā)進(jìn)一步的治療往往是徒勞的。另外有醫(yī)生更傾向于采用部分外科切除術(shù)以保留神經(jīng)功能，盡管這增加了復(fù)發(fā)的幾率并且需要進(jìn)一步的姑息性手術(shù)治療。近年來(lái)外科治療迅速發(fā)展，已經(jīng)從瘤內(nèi)刮除、部分切除發(fā)展至全椎體整塊切除。1997年，Weinstein等發(fā)表了他們的脊柱腫瘤分類分期方法。將椎體分為12個(gè)區(qū)域，A-D層。Tomita描述了他的采用線鋸對(duì)胸椎的惡性腫瘤進(jìn)行了全椎體大塊切除術(shù)。為了能夠完整切除腫瘤Boriani甚至切除了被腫瘤侵蝕的硬膜，并且采用硬膜材料進(jìn)行修補(bǔ)。外科治療現(xiàn)在是18頁(yè)\一共有28頁(yè)\編輯于星期三In2006,Borianiandhiscolleagues[1]publishedaretrospectivestudyof52chordomascasesofthemobilespineina50-yearperiod.Twelveof16caseshavingintralesionalextracapsularexcisionwithradiationhadrecurrenceataverage30months,while12of18patientshaving

enblocresectionarecontinuouslydisease-free(CDF)ataverage8years.TheynotedthattheonlytreatmentprotocolassociatedwithCDFatfollow-uplongerthan5yearsismargin-freeenblocresection.Majorcomplicationsincludevertebralarteryinjury,cerebrospinalfluid(CSF)leak,nervepalsiesandparalysis.Themortalityrateforskullbasetumorsisfrequentlyquotedat5%,andthisnumberis7.7%inBoriani’sspineseries.Inhisseriesnodifferentiationinthetypeofradiotherapyusedwasmentioned.Theyalsodidnotdiscussthedifferencebetweencervicalandthoracolumbarchordoma.Cervicaltumorexcision,especiallyintheatlantoaxialregion,mightmandatemoresophisticatedsurgicaltechnique.Inthecervicalspinefewcaseshavebeenreportedofenblocexcision,duetoanatomicalconstraints.Evenlessradicalresectioncasehaseverbeenreportedforatlantoaxialchordoma[11].Thepeculiaranatomicalcomplexityofthecervicalregionincludesthedens,thevertebralarteries,thetransverseprocessesaroundandthenerveroots.SincemostchordomasarestageIBatdiagnosis,marginfreeexcisionmeansresectionofparavertebralmuscle,nerveroot,vertebralartery,evenduraorretropharyngealmucosa.Theseseemimpossibleformostcases.

外科治療現(xiàn)在是19頁(yè)\一共有28頁(yè)\編輯于星期三2006年，Boriani及其同事發(fā)表了50年間52例脊索瘤患者的回顧性研究。16例瘤內(nèi)刮除中的12例患者平均在30個(gè)月時(shí)復(fù)發(fā)。18例椎體全切中的12例平均8年完全緩解。他們指明唯一的與無(wú)癥狀生存相關(guān)的就是沿邊界切除。主要的并發(fā)癥包括椎動(dòng)脈損傷、腦脊液漏、神經(jīng)損傷和癱瘓。顱底脊索瘤的病死率為5%，但是他們統(tǒng)計(jì)為7.7%。

在他們的研究中，未提及不同的放療類型。也沒(méi)有提及頸椎與胸腰椎脊索瘤的不同。頸椎腫瘤特別是寰樞椎腫瘤要求更復(fù)雜的外科技術(shù)。由于解剖復(fù)雜，關(guān)于頸椎腫瘤少見(jiàn)全脊椎切除術(shù)的報(bào)道。寰樞椎的全脊椎切除術(shù)更為少見(jiàn)。復(fù)雜的解剖結(jié)構(gòu)包括齒狀突、椎動(dòng)脈、橫突和神經(jīng)根。由于大部分脊索瘤是IB期，邊界外切除意味著椎旁肌肉、神經(jīng)根甚至硬膜和咽后粘膜。對(duì)于大部分病例這似乎不可能。外科治療現(xiàn)在是20頁(yè)\一共有28頁(yè)\編輯于星期三In1999,Tomitaetal.[5]firstdescribedTESforcervicalchordoma(IB)throughasingleanteriorapproachwithT-saw.Unfortunately,aftertheC5vertebralbodyhadbeenremovedwithaT-sawtheresidualtumoralongtheC6rootandduramaterhadtoberemovedinapiecemealfashion.Thetumorbedwasirrigatedbydistilledwaterandcisplatinsolutionandthenunderwentadjunctiveradiotherapy.Unfortunately,thechordomarecurred9yearslaterinthesofttissue(Tomitapersonalcommunication).In2007,Borianietal.[8]reportedenblocexcisionforaC4chordoma(IA)withcombinedposteriorandanteriorapproach.HeconcludedthatTESisfeasibleinthemidcervicalspinebydoubleapproaches,providedthetumorinvolvesonlylayersBandC,withamaximumextensiontosectors5–8.Currieretal.[4]hasasimilarcasewith9yearsfollow-up.

Therearethreemajoranteriorapproachesforatlantoaxialchordoma.Theexperienceandabilitiesofthesurgicalteamdictatesthesurgicalprocedureused.(1)BilateralhighanteriorcervicalapproachcanprovideclearvisualizationofC2vertebralbodyandbilateraltransverseprocesses,whilethedensisnotappropriatelyexposed[13].(2)Theanteriormidlinetransoralwith/withouttranspalatineapproachisforspinallesionlocatedfromthemidclivusdowntothelevelofC3andlaterallywithin2cmtoeithersideofthemidline.However,thetumorcannotberadicallyexcisedbythisapproach,ifitextendslaterallyoutsidethetransverseprocess.Menezesreportedtranspalatopharyngealapproachfor18clivuscraniocervical

chordomacasesin2001.Heusedpiecemealexcisionwithjudicioususeofcuretandadiamondburforoccipitalcondylesoratlantallateralmasstumorinvolvement.Theauthordidnotprovideradiotherapyandfollow-upinformationindetail;atleastonepatienthadnotumorprogressionfor10years.(3)Transmandibleapproachcombinedwithanteriorcervicalapproachcouldprovideidealexposurefromclivustolowercervicalspine.Thisapproachismoreaggressiveandoftenrequiresafreeflapfortheposteriorpharyngealwallreconstruction.Rhinesetal.[12]reportedthemostaggressiveTESforC2–4chordoma(IB)in2005.Theyusedarightlateralneckdissectionandatransmandiblar,circumglossal,retropharyngealexposure.

外科治療現(xiàn)在是21頁(yè)\一共有28頁(yè)\編輯于星期三在1999年，Tomita等描述了他們采用單純前路利用線鋸對(duì)頸椎脊索瘤進(jìn)行了全脊椎切除術(shù)。不幸的是在切除頸5后，頸6神經(jīng)根周圍的腫瘤和硬膜被一片片切除。腫瘤基底被用蒸餾水和順鉑溶液浸泡，術(shù)后接受化療。但是腫瘤9年后復(fù)發(fā)。2007年，Boriani描述了他們?nèi)刁w切除頸4椎體腫瘤采用聯(lián)合前后入路。他總結(jié)到，如果腫瘤位于B和C層并且5-8區(qū)采用前后聯(lián)合入路對(duì)于中段頸椎是可行的，并且同樣進(jìn)行了9年的隨訪。

前路寰樞椎脊索瘤有三種手術(shù)方法。1、雙側(cè)高位前入路可以清楚暴露頸2椎體和橫突，但是齒狀突暴露欠佳。2、前正中經(jīng)口入路經(jīng)或不經(jīng)鄂部對(duì)于從斜坡向下直至頸3或者中線以內(nèi)2厘米。但是如果腫瘤超過(guò)橫突，則不能全切。Menezes2001年報(bào)道經(jīng)咽入路切除了18例顱頸交界脊索瘤，他謹(jǐn)慎使用刮匙鉆處理枕骨髁和寰椎側(cè)塊。作者未提供詳細(xì)的放療以及隨后的治療信息。至少一名患者術(shù)后10年無(wú)腫瘤進(jìn)展。3、經(jīng)下頜入路聯(lián)合前入路可以提供理想的從斜坡至下頸椎的顯露。這個(gè)入路暴露更為廣泛，需要將咽喉壁翻開(kāi)以便重建。Rhines等2005報(bào)道采用最廣泛的暴露方法，采用右側(cè)頸部切開(kāi)，經(jīng)下頜骨、舌周、咽后入路。外科治療現(xiàn)在是22頁(yè)\一共有28頁(yè)\編輯于星期三ThetumorwasclassifiedasIB,TESseemednotfeasible.Be