髓母细胞瘤的放射治疗.ppt

髓母细胞瘤的放射治疗,曹嫣娜,概述,来源：胚胎残留的未分化的原始髓样上皮细胞。部位：第四脑室顶上的小脑蚓部。发病率：2.1/10万/年，占儿童颅内恶性肿瘤的1520%。疾病特点：恶性程度高。生长极其迅速；手术难以完整切除；肿瘤细胞易沿脑脊液播散（1646%）。,临床表现,颅内压增高：头痛、呕吐、视神经乳头水肿小脑损害：躯干性共济失调为主其它：复视、面瘫、强迫头位、头颅增大、病理反射阳性、呛咳、小脑危象、蛛网膜下腔出血脊髓转移灶症状：背部或双下肢痛、进行性加重的截瘫或四肢瘫,分级,治疗方案,标准治疗方案(“Philadelphiaprotocol”)手术放疗：术后28天内开始。化疗(VCP)：放疗中VCR1.5mg/m2/w，共8周；放疗后6周开始CCNU75mg/m2DDP75mg/m2VCR1.5mg/m2/w3w，每6周一个周期，共8个周期。,放疗剂量,低危组：CSI23.4Gy/13f+后颅窝加量至54Gy高危组：CSI36Gy/20f+后颅窝加量至54Gy,放疗技术,常规分割CSI+Boosttoposteriorfossa超分割CSI+BoosttoposteriorfossaSRTBoosttoposteriorfossa,Craniospinalirradiation(CSI):methods,俯卧位，双手置于体侧头部两侧对穿野照射全脑及上段颈髓单后野照射脊髓各野皮肤间隔1cm每照射10Gy移动一次射野以减少各野间交叉高剂量6MV-X线照射剂量(DT)：23.4Gy36Gy,1.8Gy/f,Craniospinalirradiation(CSI):dose,Prospectiverandomisedtrialofchemotherapygivenbeforeradiotherapyinchildhoodmedulloblastoma:InternationalSocietyofPaediatricOncology(SIOP)andthe(German)SocietyofPaediatricOncology(GPO)SIOPII.MedPediatrOncol25:166-178,1995,23.4GyCSI的疗效,Risk-adaptedcraniospinalradiotherapyfollowedbyhigh-dosechemotherapyandstem-cellrescueinchildrenwithnewlydiagnosedmedulloblastoma(StJudeMedulloblastoma-96):long-termresultsfromaprospective,multicentretrialVol7October2006,23.4GyCSI对智力的影响(POG-8631),JournalofClinicalOncology,Vol16,No5,pp.172328,1998,CSI:cranial-spinaljunctionsite,THECRANIAL-SPINALJUNCTIONINMEDULLOBLASTOMA:DOESITMATTER?Int.J.RadiationOncologyBiol.Phys.,Vol.44,No.1,pp.8184,1999,超分割放疗,Twice-dailyl-Gyfractionswereadministeredseparatedby4-6h.放疗剂量和射野同常规分割,SRTBoosttoposteriorfossa,POSTERIORFOSSABOOSTINMEDULLOBLASTOMA:ANANALYSISOFDOSETOSURROUNDINGSTRUCTURESUSING3-DIMENSIONAL(CONFORMAL)RADIOTHERAPYInt.J.RadiationOncologyBiol.Phys.,Vol.46,No.2,pp.281286,2000,放疗反应,急性反应：骨髓抑制、脑水肿等；远期副作用：甲低认知障碍其它：听力减退、骨骼发育障碍、周围组织损伤继发第二恶性肿瘤等。,甲低,1.HYPOTHYROIDISMINCHILDRENWITHMEDULLOBLASTOMA:ACOMPARISONOF3600AND2340cGYCRANIOSPINALRADIOTHERAPYInt.J.RadiationOncologyBiol.Phys.,Vol.53,No.3,pp.543547,20022.ThyroidDysfunctionasaLateEffectinSurvivorsofPediatricMedulloblastoma/PrimitiveNeuroectodermalTumorsAComparisonofHyperfractionatedversusConventionalRadiotherapyCancer1997;80:798804.,认知障碍,MODELINGRADIATIONDOSIMETRYTOPREDICTCOGNITIVEOUTCOMESINPEDIATRICPATIENTSWITHCNSEMBRYONALTUMORSINCLUDINGMEDULLOBLASTOMAInt.J.RadiationOncologyBiol.Phys.,Vol.65,No.1,pp.210221,2006,影响因素包括：受照射时年龄（小于3岁差）、照射范围（全脑差于部分脑照射）、照射剂量(低剂量较好）特别是后颅窝最大剂量、肿瘤部位（幕上好于后颅窝）。,联合化疗,常用方案：VCP(VCR+CCNU+DDP);“8in1”(VCR+甲强龙+CCNU+羟基脲+甲基苄肼+DDP+CTX+Ara-c);其他方案：MTX鞘内注射CTX、VCR、VP-16、CCNU、CBP等组合,Risk-adaptedcraniospinalradiotherapyfollowedbyhigh-dosechemotherapyandstem-cellrescueinchildrenwithnewlydiagnosedmedulloblastoma(StJudeMedulloblastoma-96):long-termresultsfromaprospective,multicentretrialVol7October2006,手术+放/化疗,POSTOPERATIVENEOADJUVANTCHEMOTHERAPYBEFORERADIOTHERAPYASCOMPAREDTOIMMEDIATERADIOTHERAPYFOLLOWEDBYMAINTENANCECHEMOTHERAPYINTHETREATMENTOFMEDULLOBLASTOMAINCHILDHOOD:RESULTSOFTHEGERMANPROSPECTIVERANDOMIZEDTRIALHIT91Int.J.RadiationOncologyBiol.Phys.,Vol.46,No.2,pp.269279,2000,维持化疗对6岁以上低危组更有效；新辅助化疗增加放疗的骨髓抑制从而延长治疗时间；M分期高/低龄儿预后差；手术是否有残留对预后无明显影响。,POSTOPERATIVENEOADJUVANTCHEMOTHERAPYBEFORERADIOTHERAPYASCOMPAREDTOIMMEDIATERADIOTHERAPYFOLLOWEDBYMAINTENANCECHEMOTHERAPYINTHETREATMENTOFMEDULLOBLASTOMAINCHILDHOOD:RESULTSOFTHEGERMANPROSPECTIVERANDOMIZEDTRIALHIT91Int.J.RadiationOncologyBiol.Phys.,Vol.46,No.2,pp.269279,2000,手术+化疗-方案,适用于低龄儿童、无手术残留、无转移病灶患者,手术+化疗-结果,TreatmentofEarlyChildhoodMedulloblastomabyPostoperativeChemotherapyAloneNEnglJMed2005;352:978-86.,影响预后的因素,年龄临床分级术式后颅窝生物有效剂量（BED）放疗持续时间,Onmultivariateanalysis,age3years,M0status,50GyPFBdose,radiotherapytreatmentduration50days,anduseofchemotherapycorrelatedwithbetterfreedomfromprogressionandposteriorfossacontrolrates.ProtractedRadiotherapyTreatmentDurationinMedulloblastomaAmJClinOncol(CCT)26(1):5559,2003.,影响因素的多变量分析,Onmultivariateanalysis,age3years,M0status,50GyPFBdose,radiotherapytreatmentduration50days,anduseofchemotherapycorrelatedwithbetterfreedomfromprogressionandposteriorfossacontrolrates.ProtractedRadiotherapyTreatmentDurationinMedulloblastomaAmJClinOncol(CCT)26(1):5559,2003.,影响因素的多变量分析,年龄,TimingofRadiationinChildrenWithMedulloblastoma/PNETPediatrBloodCancer2007;48:416422,CSFcytology,TimingofRadiationinChildrenWithMedulloblastoma/PNETPediatrBloodCancer2007;48:416422,手术切除范围,TimingofRadiationinChildrenWithMedulloblastoma/PNETPediatrBloodCancer2007;48:416422,后颅窝BED,TimingofRadiationinChildrenWithMedulloblastoma/PNETPediatrBloodCancer2007;48:416422,Risk-adaptedcraniospinalradiotherapyfollowedbyhigh-dosechemotherapyandstem-cellrescueinchildrenwithnewlydiagnosedmedulloblastoma(StJudeMedulloblastoma-96):long-termresultsfromaprospective,multicentretrialVol7October2006,病理及免疫组化类型,放疗持续时间,ProtractedRadi