csch治疗策略演变

海绵窦海绵状血管瘤

治疗策略的演变

Theevolutionoftreatmentstrategiesforcavernoushemangiomaincavernoussinus海绵窦海绵状血管瘤的特点罕见，海绵窦肿瘤的2-3%多数为亚洲女性起病隐匿，进展缓慢，平均病程3年血供丰富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.病理无胶质成分与脑实质内的海绵状血管瘤相似由结缔组织分隔的薄壁血管组成

血管腔内充满浆果样物血管壁缺乏弹力层和肌层症状及体征-Symptoms压迫症状头晕，头痛，眼球突出，肢体乏力，癫痫颅神经功能障碍面部麻木，眼睑下垂，视力减退，复视、眼球活动障碍内分泌紊乱月经紊乱,泌乳素升高Direct

tumorcompressionDizziness,headache,eyeprotrusion,limbweakness,epilepsyNeurologicaldeficitsFacialnumbness,ptosis,visionloss,diplopia,eyemovementdisorderEndocrinedisordersMenstrualdisorders,elevatedprolactin影像学表现磁共振特征T2WI及T2-FLAIR均匀超高信号显著均匀强化肿瘤哑铃样病变累及鞍区血管造影动脉期肿瘤无染色KangminHe,etalEurNeurol2014;72:116–124MRIcharacteristicsT2weightedultrahighuniformsignalSignificantuniformenhancementdumbbell-likeappearanceinfiltrationofthesellarregionDSA

notumorstainingonarteryphaseA：MRIT1等信号B：MRIT2均匀极高信号C：Flair均匀高信号。D：增强MRI渐进性强化E和F：DSA动脉期无染色影像学表现

鉴别诊断

脑膜瘤血管平滑肌瘤神经鞘瘤

垂体瘤……

Differentialdiagnosis

MeningiomaVascularleiomyomaNeurilemmoma

Pituitaryadenoma

……脑膜瘤神经鞘瘤皮样囊肿动脉瘤治疗方式的选择Optimumtreatmentstrategy-controversialMicrosurgicalresection(35papers，145cases）Gamaknife（22papers）Fractionatedradiationtherapy（4papers）Cyberknife（3papers）最佳治疗模式尚未确立显微外科手术(35篇，145例）伽玛刀放射外科（22篇）常规放疗（4篇）射波刀治疗（3篇）手术全切可治愈但极具挑战

术中出血

周围解剖结构复杂全切率低

仅64%术后并发症发生率高短期71%.

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

手术治疗YI-HengYin,etal.Journalofclinicalneuroscience.2013,20,128-1331150ml19/22(86.4%)8/22(36.4%)Total18(81.8%)subtotal3(13.6%)伽玛刀---GammaKnifeIn1999IwaiA40-year-oldfemaleTreatmentPartialremovalwithseverebleedingGammaknifemarginaldose12Gy，50%isodose.ResultsDramaticallydecreasedtumorsizeUnchangedcranialnervedeficits1999年Iwai40岁女性治疗部分切除导致严重出血伽玛刀12Gy，50%等剂量线效果肿瘤明显缩小颅神经症状无改善立体定向放射外科StereotacticradiosurgerySafeandeffectiveRemarkabletumorshrinkageSymptomimprovementMinimalcomplicationsGammaknifeSingle-fractionSmall-ormediumsizedCSHCyberknifeMulti-fractionsLargeorgiantCSH安全有效肿瘤体积明显缩小神经症状改善治疗相关并发症极少伽玛刀单次小或中等大小肿瘤射波刀分次大型或巨大型肿瘤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手术Radiosurgery-Metaanalysis2012年10篇相关报道59例患者资料

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

肿瘤大小9.6cm3

（1.5–51.4cm3

）

随访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肿瘤退缩

明显退缩67.8%部分退缩25.4%

不变

6.8%症状改善

无症状13例完全缓解7例部分改善28例

维持原状11例无治疗相关并发症伽玛刀治疗中小型CSH安全有效Wangxin,etal.Journalofneuro-oncology.2012,107,239-245Tumorshrinkage

remarkable67.8%partial25.4%nochange6.8%Symptomchangesnocranialnerveimpairments13patientscompleteresolution7patientsimprovement28patientsunchanged11patientsNotreatment-relatedcomplicationsGKissafeandeffectiveforsmallormediumsizedCSH华山医院射波刀治疗中心颅底组织放射剂量耐受性动眼神经（III）---<16Gy？滑车神经（IV）

---<18Gy外展神经（VI）

---<18Gy眼支（V1）

---<16Gy？上颌支（V2）

---<16Gy？视交叉（II）

---8-10Gy垂体---15-160Gy颈内动脉---<20Gy？面神经---12-16Gy后组颅神经---<14Gy脑干---12-20Gy耳蜗---<4.5Gy垂体漏斗部远端---<17Gy颅神经对射线的耐受性研究：特殊感觉神经<躯体感觉神经<运动神经我院经验伽玛刀-中小型CSH53例（2007-2012）

女38例，男15例

平均年龄52岁

肿瘤体积13.2cm3（1–41cm3）

随访34月(2–73月).肿瘤退缩

半年后平均退缩60.2%（16.5-89.2%）

55%患者肿瘤退缩>80%症状改善33例改善

2例加重未产生与治疗相关的新症状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鞍内为主型diplopiadiplopia,blurredvision鞍旁为主型headache混合型患者女性，61岁，头晕发现CHCS伽玛刀治疗后头晕好转，21个月后复查MRI示肿瘤缩小。GKS21月后患者女性，47岁，复视及视物模糊发现CHCS伽玛刀治疗后1月症状消失，45个月后复查MRI示肿瘤缩小GKS45月后患者女性，40岁，复视和外展麻痹发现CHCS伽玛刀治疗后1月复视消失，46个月后复查MRI示肿瘤缩小GKS46月后大型CSHII期前瞻性研究肿瘤体积>20cm3，21Gy/3Fx14例肿瘤体积50.2cm3（22.6–96.3cm3）

随访15月(6–36月)肿瘤平均退缩77%（44-99%）6例治疗前有颅神经症状1例缓解5例改善未产生与治疗相关的新症状Wangxin,etal.Internationjournalofradiationoncologybiologyphysics.2012,157,961-969.我院经验OurexperienceLargeCSHPhaseIIstudyTumorvolume>20cm3，21Gy/3Fx14patientsTumorvolume50.2cm3（22.6–96.3cm3）Followup15m(6–36m)Tumorshrinkage77%（44-99%）6patientswithcranialnerveimpairments

completeresolution1patientsimprovement5patientsNotreatment-relatedcomplications巨大型CSHII期前瞻性研究肿瘤体积>40cm3，18-22Gy/3-4Fx31例肿瘤体积64.4cm3

(40.9-145.3cm3)

随访30月(6–78月)肿瘤平均退缩88.1%(62.3%-99.4%)30例治疗前有颅神经症状19例完全缓解11例改善副作用

轻度头痛10例

呕吐5例癫痫1例Wangxin,etal.Journalofneurosurgery.Accepted我院经验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照射剂量Dose肿瘤控制TumorresponseSymptomchangesSummaryofpost-treatmentchangesinsymptomsSymptomspretreatmentCranialnervedeficitsHeadacheDizzyNauseaSuppressedmenstruationWalkinginstabilityHandnumbnessNo.deficitsTotal(%)ⅡⅢⅣⅤⅥno.ofpatients1410244992231131Recovery610233782131019(61.3%)Improved80011210100011(35.5%)Averagetimetorecovery(months)622638621033大型CSH三年以上长期结果40例肿瘤体积

48.6cm3(11.3-140.1cm3)

随访53月(36–90月)肿瘤平均退缩85%(41%-100%)30例有颅神经症状19例完全缓解11例改善副作用

轻度头痛10例

呕吐5例癫痫1例我院经验OurexperienceLong-termoutcomesoflargeCSH40casesTumorvolume48.6cm3(11.3-140.1cm3)Followup53months(36–90months)Tumorshrinkage85%(41%-100%)30patientswithcranialnerveimpairments

completeresolution19patientsimprovement11patientsTreatment-relatedcomplicationsmildheadache10patientsvomiting5patientsseizure1patient大型CSH三年以上长期结果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例手术后残留或复发男26例；女76例年龄：51（26-80）肿瘤最大直径：40mm（23-75）肿瘤体积：39.1cm3

（2.9-145.1）患者资料2008.1-2014.12102patients

Residualorrecurrenceaftersurgeryin14cases

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

（2.9-145.1）剂量方案DosescheduleTumorvolumeDoseschedule<513.0Gy/1fx5≤V<1017.8-19.0Gy/2fx10<V≤4018.4-21.0Gy/3fx>4018.0-22Gy/4fxTheequivalentofGammaknife10-13Gy/1fxdosecurving华山医院射波刀治疗中心dosecurving7mafterCK18mafterCK45mafterCKTumorvolume13.5cm3

21Gy/3FxBeforeCK12mafterCKTumorshringkage95％Treatmentplan4yearsafterCKMRIFlairMRIT2BeforeCK7mafterCKTumorshrinkage95%2yafterCK

Tumorshrinkage99%1y1y2

y2

yTumordiameter6.3cmTumorvolumn80cm3BeforeCK2yafterCK4yafterCK7mafterCKTumordiameter7.5cmTumorvolume140cm37mafterCK

tumorshrinkage75%3yafterCK

tumorshrinkage90%肿瘤生长速度1998