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纵隔肿瘤放射治疗 复旦大学附属肿瘤医院放疗科吴开良 2003 04 22 纵隔应用解剖 纵隔分区及不同肿瘤的好发部位 纵隔肿瘤发病情况 1 来源各异 大多为良性 恶性占10 1 25 儿童恶性占50 神经源性肿瘤 畸胎类肿瘤 胸腺肿瘤占2 3上 前 中 后纵隔的肿瘤分布分别为20 20 20 和30 儿童63 位于后纵隔 纵隔肿瘤发病情况 2 神经源性肿瘤31627 1 畸胎类肿瘤30826 4 胸腺瘤及囊肿24120 7 各类囊肿988 4 胸内甲状腺736 3 其他13011 1 总计1166100 纵隔肿瘤影像检查 胸片是基本的手段X线胸部透视 转动身体 食管钡剂CT纵隔肿瘤最常用的检查方法MRI的价值 分辨率高 多向性 能够分辨血管和纵隔淋巴结 判断后纵隔肿瘤是否侵犯椎管 CTscanimageclearlyillustratesthemassintherightanterolateralmediastinum 纵隔肿瘤的有创性检查 明确诊断并作为治疗 活检或切除针吸活检 简单有效 不能够切除者可获得非手术治疗的机会 不适用于淋巴瘤纵隔镜 适用于气管前 气管旁 左侧无名静脉 右侧支气管区肿块左下气管旁和胸骨后肿块可用胸骨旁纵隔镜胸腔镜 可用于后纵隔肿瘤 纵隔肿瘤诊断 大部分病例需要手术或穿刺活检诊断时候应该考虑1 肿瘤在纵隔内还是肺内或胸膜腔内2 是良性还是恶性3 适宜用那种治疗方法 纵隔肿瘤 肺内肿块 瘤体中心在纵隔内瘤体和纵隔不可分不随呼吸运动咳嗽动作固定基底径为最大径有胸膜反折与肺裂无关 跨叶 不同呼吸状态下肿块与肺裂的关系可变 肺内瘤体和纵隔可分与肺纹理方向一致运动咳嗽动作向肺门运动基底径小于肿瘤最大径无胸膜反折跨叶少见不同呼吸状态下肿块与肺裂的关系可变 纵隔肿块和肺内肿块的鉴别 纵隔肿瘤良恶性鉴别 良性 恶性生长速度慢快肿瘤轮廓边缘规则边缘模糊毛刺骨改变骨吸收 萎缩浸蚀性破坏上腔静脉压迫无有远地转移无有 纵隔肿瘤治疗原则 手术治疗 无手术禁忌症的大多外科治疗放射治疗单纯放疗 试探性放疗 前中纵隔巨大肿瘤压迫症状明显而不适宜手术的病人 20Gy 2周 姑息性放疗 晚期病人解除痛苦缓解压迫20 40Gy 2 4周 根治性放疗 淋巴肉瘤及不适宜手术的胸腺瘤及生殖细胞瘤 45 60Gy 4 5 6周手术前放疗 较少用手术后放疗 浸润性胸腺瘤及生殖细胞瘤术后及恶性纵隔肿瘤术后残留手术后2 4周进行 45 60Gy 4 5 6周 鉴别诊断 纵隔恶性淋巴瘤 中心型肺癌 胸椎结核并发椎旁脓肿 纵隔转移性肿瘤 纵隔淋巴结核 胸主动脉瘤 食管平滑肌瘤 纵隔巨大淋巴结增生 其他 膈疝 食管扩张 包裹性胸腔积液肺内囊肿 结节病 14 胸腺瘤 Thymoma 发病情况 Accountfor17 30 ofanteriormediastinaltumours Theincidenceis0 18per100 000formenand0 10per100 000forwomen 胸腺瘤 病理 前纵隔最常见的肿瘤 中年发病最高传统分型 上皮细胞为主型 Hassall小体 淋巴细胞为主型 混合细胞型 梭型细胞良性 包膜完整 手术后不复发恶性 30 包膜不完整 肿瘤局部突破包膜侵犯临近组织良恶性都可有可有囊性变 出血和钙化胸腺癌 上皮细胞癌 罕见 Anatomyofthethymuswithemphasisonthebloodsupplyandrelationtorecurrentlaryngealandphrenicnerves illustrationcourtesyofHenryStiller KeeslerMedicalCenter Biloxi Mississippi Lateralview Thethymicarteriesarederivedfromtheadjacentinternalmammaryarteriesandtheinferiorthymicveinemptiesintotheinnominatevein Thesesurroundingvascularandneuralstructuresmaybeinvadedduringthespreadofthymoma illustrationcourtesyofHenryStiller KeeslerMedicalCenter Biloxi Mississippi 胸腺瘤 临床表现 40 50岁年龄组发病最高多数患者无症状 偶尔在X线检查发现肿块对临近纵隔结构侵犯和压迫症状咳嗽 胸痛 气急 声音嘶哑 上腔V压迫 胸水 上肢疼痛 咳毛发或豆渣样物 重症肌无力 35 40 单纯红细胞性贫血5 低丙种球蛋白血症5 15 甲状腺毒性病 风湿性关节炎 系统性红斑狼疮 Cushing综合症 HistologicalClassificationofThymoma 8 4 0 TypeA068 34 0 TypeAB017 8 5 TypeB11 22months 39 19 5 TypeB275 5y27 13 5 TypeB370 5y36 18 0 TypeC48 0 5yhistologicsubtypes logranktest P 0 001 MasaokaStageI 26 13 0 wereinStageII 65 32 5 wereinStageIII and13 6 5 wereinStageIV Stagewashighlysignificantinpredictingsurvival logrank testP 0 001 Theassociationbetweenhistologicsubtypeandinvasivebehavior stage wasstatisticallysignificant P 0 001 HistologywasanindependentpredictivefactorofsurvivalinStageIandIIthymomas Thirtypatients 15 0 presentedwithMG MGwassignificantlymorefrequentinTypeB2andB3thaninTypeA AB andB1thymomas P 0 01 Onmultivariateanalysis MGhadnoadverseeffectonsurvival P 0 17 Radiationorchemotherapyimprovedpatients survivalat5and10yearsinTypeB2 B3 andCthymomas logranktest P 0 003 GangChen Cancer2002 95 420 9 胸腺瘤 分期系统 传统分期 1978年Bergh分期I期 局限在包膜内II期 包膜侵犯 周围脂肪组织或纵隔受侵犯III期 侵犯临近的器官如心包 大血管和肺组织或胸内转移 MasaokaStagingSystemofThymomasandCorrespondingTherapy MasaokaA Follow upstudyofthymomaswithspecialreferencetotheirclinicalstages Cancer1981 48 2485 2492 53patients twodoctor2withtypeA 14withtypeAB ninewithtypeB1 14withtypeB2 fourwithtypeB3 andeightwithtypeCepithelialtumors smoothcontoursonCT 4 4 100 roundshapes 3 5 4 88 thananyothertypeofthymicepithelialtumor all p 0 05 TypeCtumorshadahigherprevalenceofirregularcontours 6 8 75 all p 0 05 CalcificationwasmorefrequentlyseenintypeB1 4 9 44 typeB2 8 5 14 61 andtypeB3 3 4 75 tumorsthanintypeAB 2 14 14 andtypeC 0 5 8 6 tumors all p 0 05 SmoothcontoursandaroundshapearemostsuggestiveoftypeAthymicepithelialtumor whereasirregularcontoursaremostsuggestiveoftypeCtumor CalcificationissuggestiveoftypeBtumors CTisoflimitedvalue however indifferentiatingtypeAB B1 B2 andB3tumors TomiyamaN AJRAmJRoentgenol2002Oct 179 4 881 6 CTfeaturesofvarioussubtypesand1999WHO 胸腺瘤 治疗选择 肿瘤局限无广泛胸内外转移者以手术治疗为首选浸润性胸腺瘤应术后放疗非浸润性胸腺瘤不常规术后放疗 但应该密切观察 复发者应该给再手术加放疗晚期的胸腺瘤 局部放疗 化疗 MasaokaStagingSystemofThymomasandCorrespondingTherapy PracticeGuidelines StageICompleteresectionofawell encapsulated non invasivethymomaisusuallycurative withariskoflocalrecurrenceoflessthan2 Inpatientswithmyastheniagravis operativemortalitycannowbeminimisedwithcloseattentiontorespiratorysupportwhenplanningsurgicaltreatment Thereisnoroleforradiotherapyunlessanon invasivethymomaisincompletelyresected StageIISurgeryEnblocsurgicalresectionofthetumourwithintactcapsuleisstandard Thesurgeonmustcarefullyassessthetumourduringtheoperationinanattempttoidentifyanyinvasivequalities andshouldmarkareasthataresuspiciousforinvasioninanefforttoguidethepathologist smicroscopicdescriptionandtheradiationoncologist sadjuvanttherapy RadiotherapyThevalueofadjuvantradiationtherapyininvasivethymomasiswelldocumentedandshouldbeincludedinthetreatmentregimenregardlessofthecompletenessoftumourresection Inareviewof115completelyresectedinvasivethymomas Curranetal 1988 arecurrencerateof5 wasdocumentedwhenadjuvantradiationtherapywasemployed thisincreasedto28 withoutadjuvantradiotherapy ChemotherapyAdjuvantchemotherapyhasnotbeenreportedinstageIIthymoma Radiotherapyisthetreatmentofchoicewhentheareaatriskofrecurrencecanbeencompasseswithinareasonableradiotherapyvolume Incaseswherethesurgeonidentifiespleuralinvasion StageIIB suchthatthepleuralspaceisjudgedtobeatriskofdropmetastases theareaatriskextendsoutsideareasonableradiotherapyvolume Insuchcases adjuvantchemotherapymaybeaconsiderationparticularlyinyoungorfitpatients Complexcasesareidealformulti disciplinaryreviewatlungconference StageIIISurgeryWheninvasionofneighbouringorgansisidentifiedintra operatively aggressiveresectionincludinglung pleura phrenicnerve pericardium andgreatvesselsisoccasionallynecessary Adjuvantthoracicirradiationmaybeguidedifareasathighriskofrecurrencearemarkedwithsurgicalclips Theroleofsubtotalresectionordebulkingproceduresinadvanced unresectablestageIIIdiseaseisuncertain Wheninvasionofadjacentorgansisidentifiedbeforesurgery preoperativedownstagingwithchemotherapyorchemoradiationshouldbeconsidered Afterpre operativetherapy definitivelocalsurgeryshouldbeperformed4to6weeksafterthefinalcycleofchemotherapy ChemotherapyDuringthepastdecade thymomahasclearlybeenidentifiedasachemosensitivetumour Theoptimalregimenandpreciseroleofchemotherapycontinuestobeuncertainbecausethelowincidenceofthymomaslimitsfeasibilityoflargeclinicaltrials Cisplatincontainingcombinationchemotherapyregimensappeartobethemostactive ThePACregimenconsistingofcisplatin doxorubicin andcyclophosphamide Loehreretal 1994 andetoposidepluscisplatin Giacconeetal 1996 arecapableofinducingresponsesinthemajorityofpatientswithadvanceddiseasewithsomecompleteresponses NeoadjuvantchemotherapywithPACbeforeresectionoflocallyadvancedcasesisassociatedwithahighresponseratebutthemajorityofpatientshadhistologicallyviabletumourintheresectionspecimenandreceivedpost operativeradiotherapy Reaetal 1993 Theoretically riskoftumourdisseminationoutsidethemediastinumatthetimeofsurgeryshouldbediminishedifahigherproportionofpatientsweredownstagedtopT0 AhigherpT0rateshouldbepossiblewhencombinedchemoradiationisgivenbeforesurgery PreoperativethoracicirradiationgivenconcurrentlywithcisplatinplusetoposidehasbeenwidelyusedinthecombinedmodalitytherapyofstageIIINSCLC Albainetal 1995 withacceptableriskoftoxicityandpromisingresults AsimilarmodeloftreatmentmaybesuitableforstageIIIthymomawheninitialcompleteresectionappearsunlikely RadiotherapyAdjuvantradiotherapyafterresectionisstandardincaseswheretheriskforrecurrenceisjudgedtoresidewithinareasonableradiotherapyvolume ThoracicirradiationdeliveredconcurrentlywithcisplatinplusetoposideshouldbedeliveredinthesamefashiondescribedforStageIIINSCLC provideappropriatelungcancersection StageIVandRecurrentThymomaChemotherapyEtoposidepluscisplatinorthePACregimeninduceresponsesinmorethanhalfofadvancedcaseswithmediansurvivalof3 4yearsand5 yearsurvivalof20 30 Concurrentchemoradiationshouldnotbeconsideredforpatientswithdiseasenotencompassablewithinareasonableradiotherapyvolume SurgeryandRadiotherapySurgerymaybeconsideredinStageIVAthymomasthatrespondwelltoinitialchemotherapy Suchcasesareappropriateforlungconference ThoracicirradiationasaconsolidativetherapyaftergoodchemotherapyresponseofstageIVthymomasshouldbeconsideredonacasebycasebasis Recurrentthymomathatisresistanttochemotherapymaybeappropriateforpalliativeradiotherapy 胸腺瘤 放射治疗 放射源 高能x线 钴 60或电子线照射范围 局部瘤床 1cm边界心包转移或心包积液 全心包 全纵隔3000 3500cGy 3 3 5周后局部瘤床加量胸膜或肺转移 半胸或者全胸照射1500 2000cGy 2 3周后局部瘤床加量放射剂量 淋巴细胞为主型 5000cGy 5周上皮细胞为主型 6000 7000cGy 6 7周设野 可以用高能X线和电子线的配合 一般多用两前斜野或两前斜野加后野 Fornasieroandcolleagues 1991 Reportedsuccessfulcasesandsomelong termsurvivorscisplatin vincristine doxorubicin cyclophosphamideforincompletelyresectedinvasivethymomasorcaseswithunresectabledisease In32patients a47 completeand90 overallresponseratewasnotedwithamediansurvivaltimeof15months 胸腺瘤 重症肌无力的处理 外科手术或放疗时应该慎重 治疗前用吡啶斯的明60mg tid 或者新斯的明0 5mg肌注 50 横纹肌抗体阳性 该抗体无种属特异性缓慢停药 3 6个月 出现加重应该调整剂量并用激素必要时用呼吸机用药期间出现付交感神经兴奋症状可用阿托品缓解禁用箭毒类肌松剂放疗从小剂量开始 100cGy f 缓慢加量 胸腺瘤 失败原因和预后因素 局部失败是主要的原因 远地转移 主要的部位为肺 肝 骨预后因素 肿瘤的浸润性浸润性胸腺瘤非浸润性胸腺瘤可切除性100 58 局部复发率0 3 8 20 5年生存率85 100 33 55 病理类型 上皮型更差肌无力 不是影响预后的因素手术的完整程度其他 年龄 儿童差 单纯红细胞贫血 获得性丙球缺乏 病期 SurvivalofThymomabyStage TheMemorialSloanKetteringExperience TomohiroMurakawaAsianCardiovascThoracAnn2002 10 150 154166patientsincludedinthestudy 6 3 6 hadassociatedPRCAand61 36 7 hadassociatedMGThreeofthepatientswithPRCAhadastageIthymoma 1patienthadastageIIIthymoma and2patientshadastageIVathymoma In3ofthese thepurered cellaplasiaappearedaftersurgicalintervention Remissionwasobservedin2patientswhounderwentextendedthymectomy Theother4patientssubsequentlydiedfrompurered cellaplasia Theoutcomeinpatientswithpurered cellaplasiawaspoorerthanthatintheentiregroupofpatientswiththymomaandinthosewiththymomacomplicatedbymyastheniagravis Thepossibleonsetofpurered cellaplasiaafterthyme

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