海綿竇海綿狀血管瘤

治療策略的演變

Theevolutionoftreatmentstrategiesforcavernoushemangiomaincavernoussinus海綿竇海綿狀血管瘤的特點罕見，海綿竇腫瘤的2-3%多數(shù)為亞洲女性起病隱匿，進展緩慢，平均病程3年血供豐富Extremelyrare,2%to3%ofCStumorsPredominanceinMiddleagedwomenInsidiousonset,slowprogression,meandiseasedurationof3yearspropensityforprofusebleeding

Pathology:

truevascularneoplasmsTypeA:sponginess-shapedwithanintactpseudocapsuleTypeB:mulberry-shapedw/wtthepseudocapsuleMorphologicalsubtypes(byZhouetalfromHuashanHospital)ChenL,ZhouLF,

etal.SurgNeurol,2003;60:31-8.ChenL,ZhouLF,etal.SurgNeurol,2003;60:31-8.AABB

ChuangCC,etal.JofClinicalNeuroscience,2006;13:672-5.病理無膠質成分與腦實質內的海綿狀血管瘤相似由結締組織分隔的薄壁血管組成

血管腔內充滿漿果樣物血管壁缺乏彈力層和肌層癥狀及體征-Symptoms壓迫癥狀頭暈，頭痛，眼球突出，肢體乏力，癲癇顱神經(jīng)功能障礙面部麻木，眼瞼下垂，視力減退，復視、眼球活動障礙內分泌紊亂月經(jīng)紊亂,泌乳素升高Direct

tumorcompressionDizziness,headache,eyeprotrusion,limbweakness,epilepsyNeurologicaldeficitsFacialnumbness,ptosis,visionloss,diplopia,eyemovementdisorderEndocrinedisordersMenstrualdisorders,elevatedprolactin影像學表現(xiàn)磁共振特征T2WI及T2-FLAIR均勻超高信號顯著均勻強化腫瘤啞鈴樣病變累及鞍區(qū)血管造影動脈期腫瘤無染色KangminHe,etalEurNeurol2014;72:116–124MRIcharacteristicsT2weightedultrahighuniformsignalSignificantuniformenhancementdumbbell-likeappearanceinfiltrationofthesellarregionDSA

notumorstainingonarteryphaseA：MRIT1等信號B：MRIT2均勻極高信號C：Flair均勻高信號。D：增強MRI漸進性強化E和F：DSA動脈期無染色影像學表現(xiàn)

鑒別診斷

腦膜瘤血管平滑肌瘤神經(jīng)鞘瘤

垂體瘤……

Differentialdiagnosis

MeningiomaVascularleiomyomaNeurilemmoma

Pituitaryadenoma

……腦膜瘤神經(jīng)鞘瘤皮樣囊腫動脈瘤治療方式的選擇Optimumtreatmentstrategy-controversialMicrosurgicalresection(35papers，145cases）Gamaknife（22papers）Fractionatedradiationtherapy（4papers）Cyberknife（3papers）最佳治療模式尚未確立顯微外科手術(35篇，145例）伽瑪?shù)斗派渫饪疲?2篇）常規(guī)放療（4篇）射波刀治療（3篇）手術全切可治愈但極具挑戰(zhàn)

術中出血

周圍解剖結構復雜全切率低

僅64%術后并發(fā)癥發(fā)生率高短期71%.

晚期18%CurativepotentiallybutextremelychallengingSevereintraoperativehemorrhageComplicatedneurovascularstructuresLowtotalremovalrateOnly64%HighPostoperativecranialneuropathiesratePostoperativecomplications71%LTmorbidity18%SurgeryFraserJF,etal.Skullbase.2008Sep;18(5):309-15.

手術治療YI-HengYin,etal.Journalofclinicalneuroscience.2013,20,128-1331150ml19/22(86.4%)8/22(36.4%)Total18(81.8%)subtotal3(13.6%)伽瑪?shù)?--GammaKnifeIn1999IwaiA40-year-oldfemaleTreatmentPartialremovalwithseverebleedingGammaknifemarginaldose12Gy，50%isodose.ResultsDramaticallydecreasedtumorsizeUnchangedcranialnervedeficits1999年Iwai40歲女性治療部分切除導致嚴重出血伽瑪?shù)?2Gy，50%等劑量線效果腫瘤明顯縮小顱神經(jīng)癥狀無改善立體定向放射外科StereotacticradiosurgerySafeandeffectiveRemarkabletumorshrinkageSymptomimprovementMinimalcomplicationsGammaknifeSingle-fractionSmall-ormediumsizedCSHCyberknifeMulti-fractionsLargeorgiantCSH安全有效腫瘤體積明顯縮小神經(jīng)癥狀改善治療相關并發(fā)癥極少伽瑪?shù)秵未涡』蛑械却笮∧[瘤射波刀分次大型或巨大型腫瘤517571%36/5173.3%55/753750Resolved22%Improved46%Stable32%Resolved48%Improved13%Stable36%New15%YI-HengYin,etal.Journalofclinicalneuroscience.2013,20,128-133放射外科vs手術Radiosurgery-Metaanalysis2012年10篇相關報道59例患者資料

女37/59(62.7%)男22/59(37.3%)49.2歲(14–78)

腫瘤大小9.6cm3

（1.5–51.4cm3

）

隨訪49.2月(6–156月)Wangxin,etal.Journalofneuro-oncology.2012,107,239-245201210

papers59patientsclinical

materials37(62.7%)femaleand22(37.3%)male

49.2years(range14-78years)meantumorsize9.6cm3

（1.5-51.4cm3）Meanfollow-up:49.2monthsRadiosurgery-Metaanalysis腫瘤退縮

明顯退縮67.8%部分退縮25.4%

不變

6.8%癥狀改善

無癥狀13例完全緩解7例部分改善28例

維持原狀11例無治療相關并發(fā)癥伽瑪?shù)吨委熤行⌒虲SH安全有效Wangxin,etal.Journalofneuro-oncology.2012,107,239-245Tumorshrinkage

remarkable67.8%partial25.4%nochange6.8%Symptomchangesnocranialnerveimpairments13patientscompleteresolution7patientsimprovement28patientsunchanged11patientsNotreatment-relatedcomplicationsGKissafeandeffectiveforsmallormediumsizedCSH華山醫(yī)院射波刀治療中心顱底組織放射劑量耐受性動眼神經(jīng)（III）---<16Gy？滑車神經(jīng)（IV）

---<18Gy外展神經(jīng)（VI）

---<18Gy眼支（V1）

---<16Gy？上頜支（V2）

---<16Gy？視交叉（II）

---8-10Gy垂體---15-160Gy頸內動脈---<20Gy？面神經(jīng)---12-16Gy后組顱神經(jīng)---<14Gy腦干---12-20Gy耳蝸---<4.5Gy垂體漏斗部遠端---<17Gy顱神經(jīng)對射線的耐受性研究：特殊感覺神經(jīng)<軀體感覺神經(jīng)<運動神經(jīng)我院經(jīng)驗伽瑪?shù)?中小型CSH53例（2007-2012）

女38例，男15例

平均年齡52歲

腫瘤體積13.2cm3（1–41cm3）

隨訪34月(2–73月).腫瘤退縮

半年后平均退縮60.2%（16.5-89.2%）

55%患者腫瘤退縮>80%癥狀改善33例改善

2例加重未產(chǎn)生與治療相關的新癥狀XuqunTang,etal.ActaNeurochir.2015,157,961-969.GK-smallormediumsizedCSH53patients（2007-2012）

Female38patients，Male15patients52yearstumorvolume13.2cm3（1–41cm3）followup34m(2–73m).Tumorshrinkage60.2%（16.5-89.2%）>80%in55%patientsSymptomchanges

improvement28patientsaggravation2patientsNotreatment-relatedcomplicationsblurredvisiondizziness.fatiguedizziness鞍內為主型diplopiadiplopia,blurredvision鞍旁為主型headache混合型患者女性，61歲，頭暈發(fā)現(xiàn)CHCS伽瑪?shù)吨委熀箢^暈好轉，21個月后復查MRI示腫瘤縮小。GKS21月后患者女性，47歲，復視及視物模糊發(fā)現(xiàn)CHCS伽瑪?shù)吨委熀?月癥狀消失，45個月后復查MRI示腫瘤縮小GKS45月后患者女性，40歲，復視和外展麻痹發(fā)現(xiàn)CHCS伽瑪?shù)吨委熀?月復視消失，46個月后復查MRI示腫瘤縮小GKS46月后大型CSHII期前瞻性研究腫瘤體積>20cm3，21Gy/3Fx14例腫瘤體積50.2cm3（22.6–96.3cm3）

隨訪15月(6–36月)腫瘤平均退縮77%（44-99%）6例治療前有顱神經(jīng)癥狀1例緩解5例改善未產(chǎn)生與治療相關的新癥狀Wangxin,etal.Internationjournalofradiationoncologybiologyphysics.2012,157,961-969.我院經(jīng)驗OurexperienceLargeCSHPhaseIIstudyTumorvolume>20cm3，21Gy/3Fx14patientsTumorvolume50.2cm3（22.6–96.3cm3）Followup15m(6–36m)Tumorshrinkage77%（44-99%）6patientswithcranialnerveimpairments

completeresolution1patientsimprovement5patientsNotreatment-relatedcomplications巨大型CSHII期前瞻性研究腫瘤體積>40cm3，18-22Gy/3-4Fx31例腫瘤體積64.4cm3

(40.9-145.3cm3)

隨訪30月(6–78月)腫瘤平均退縮88.1%(62.3%-99.4%)30例治療前有顱神經(jīng)癥狀19例完全緩解11例改善副作用

輕度頭痛10例

嘔吐5例癲癇1例Wangxin,etal.Journalofneurosurgery.Accepted我院經(jīng)驗OurexperienceGiantCSHPhaseIIstudyTumorvolume>40cm3，18-22Gy/3-4Fx31patientsTumorvolume64.4cm3（40.9–145.3cm3）Followup30m(6–78m)Tumorshrinkage88.1%(62.3%-99.4%)30patientswithcranialnerveimpairments

completeresolution19patientsimprovement11patientsTreatment-relatedcomplicationsmildheadache10patientsvomiting5patientsseizure1patientSummaryofdosingschedule，biologicallyequivalentdosesandsingle-doseequivalentsDosingscheduleBED

Totaldose(Gy)No.offractionsα/β=3Single-doseequivalent(Gy)No.ofpatients%Prescribeddoseoftumor18445.0010.22516.120453.3311.2326.520.8456.911.6526.519.5361.7512.2013.222462.3312.251135.520.4366.6012.7113.221370.0013.00929.0DmaxofOpticnerve(mean)13.70334.568.801135.615.22434.528.792064.5Dmaxofopticchiasm(mean)15.86343.8110.061135.616.34438.599.362064.5Dmaxofbrainstem(mean)19.08359.5311.951135.620.10453.7711.292064.5照射劑量Dose腫瘤控制TumorresponseSymptomchangesSummaryofpost-treatmentchangesinsymptomsSymptomspretreatmentCranialnervedeficitsHeadacheDizzyNauseaSuppressedmenstruationWalkinginstabilityHandnumbnessNo.deficitsTotal(%)ⅡⅢⅣⅤⅥno.ofpatients1410244992231131Recovery610233782131019(61.3%)Improved80011210100011(35.5%)Averagetimetorecovery(months)622638621033大型CSH三年以上長期結果40例腫瘤體積

48.6cm3(11.3-140.1cm3)

隨訪53月(36–90月)腫瘤平均退縮85%(41%-100%)30例有顱神經(jīng)癥狀19例完全緩解11例改善副作用

輕度頭痛10例

嘔吐5例癲癇1例我院經(jīng)驗OurexperienceLong-termoutcomesoflargeCSH40casesTumorvolume48.6cm3(11.3-140.1cm3)Followup53months(36–90months)Tumorshrinkage85%(41%-100%)30patientswithcranialnerveimpairments

completeresolution19patientsimprovement11patientsTreatment-relatedcomplicationsmildheadache10patientsvomiting5patientsseizure1patient大型CSH三年以上長期結果Table2Clinicalresponseaftermulti-fractionSRSforCSHsPre-treatmentsymptomsandsignsResponseNo:(%)No.Completeresolved:NoImprovement:No.Visualimpairment146(42%)8(58%)Diplopia65(83.3%)Facialnumbness77(100%)Slightheadache1211(91.6)Dizziness77(100%)Ptosis119(81.8)1(9%)Amennorrhea33(100%)Exophthalmos11(100%)Decreasinghearing1noNumbnessofcontralaterallimbs11(100%)Patients

2008.1-2014.12102例14例手術后殘留或復發(fā)男26例；女76例年齡：51（26-80）腫瘤最大直徑：40mm（23-75）腫瘤體積：39.1cm3

（2.9-145.1）患者資料2008.1-2014.12102patients

Residualorrecurrenceaftersurgeryin14cases

Male25.5%；Female74.5%Age：51（26-80）Tumordiameter：40mm（23-75）Tumorvolume：39.1cm3

（2.9-145.1）劑量方案DosescheduleTumorvolumeDoseschedule<513.0Gy/1fx5≤V<1017.8-19.0Gy/2fx10<V≤4018.4-21.0Gy/3fx>4018.0-22Gy/4fxTheequivalentofGammaknife10-13Gy/1fxdosecurving華山醫(yī)院射波刀治療中心dosecurving7mafterCK18mafterCK45mafterCKTumorvolume13.5cm3

21Gy/3FxBeforeCK12mafterCKTumorshringkage95％Treatmentplan4yearsafterCKMRIFlairMRIT2BeforeCK7mafterCKTumorshrinkage95%2yafterCK

Tumorshrinkage99%1y1y2

y2

yTumordiameter6.3cmTumorvolumn80cm3BeforeCK2yafterCK4yafterCK7mafterCKTumordiameter7.5cmTumorvolume140cm37mafterCK

tumorshrinkage75%3yafterCK

tumorshrinkage90%腫瘤生長速度1998