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1、髓母细胞瘤的放射治疗髓母细胞瘤的放射治疗曹嫣娜概述概述来源: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分级Stage Risk staging system Stage Changs M stagin
2、g systemLow-risk Localized disease at the time of diagnosis M0 No evidence of gross subarachnoid orGroup Age 3 years hematogenous metastasis Total tumor resection or subtotal with residual tumor 1.5 cm3 High-risk Disseminated disease at the time of diagnosis M1 Microscopic tumor cells found inGroup
3、cerebrospinal fluid Age 3 years M2 Gross nodule seeding seen in the cerebellar or cerebral subarachnoid space or in the third or lateral ventricles Subtotal tumor resection with a residual tumor M3 Gross nodule seeding in the spinal 1.5 cm3 subarachnoid space metastasis M4 Extraneural治疗方案标准治疗方案(“Phi
4、ladelphia protocol”)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/m2 DDP75mg/m2 VCR1.5mg/m2/w3w,每6周一个周期,共8个周期。放疗剂量低危组:CSI 23.4Gy/13f+后颅窝加量至 54Gy高危组:CSI 36Gy/20f+后颅窝加量至54Gy放疗技术常规分割CSI+Boost to posterior fossa 超分割CSI+Boost to posterior fossa SRT Boost to posterior fossaCraniospinal irradi
5、ation(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/fCraniospinal irradiation(CSI):doseradiotherapy alone(5-year EFS)Chemotherapy+(5-year EFS)standard radiotherapy reduced-dose radiotherapy60%7.8%41%8%75%7%69%8%Prospective randomised tr
6、ial of chemotherapy given before radiotherapy in childhood medulloblastoma:International Society of Paediatric Oncology(SIOP)and the(German)Society of Paediatric Oncology(GPO)SIOP II.Med Pediatr Oncol 25:166-178,1995 23.4GyCSI的疗效Risk-adapted craniospinal radiotherapy followed by high-dose chemothera
7、py and Risk-adapted craniospinal radiotherapy followed by high-dose chemotherapy and stem-cell rescue in children with newly diagnosed medulloblastoma(St Jude stem-cell rescue in children with newly diagnosed medulloblastoma(St Jude Medulloblastoma-96):long-term results from a prospective,multicentr
8、e trialMedulloblastoma-96):long-term results from a prospective,multicentre trial Vol 7 October 2006Vol 7 October 200623.4GyCSI对智力的影响(POG-8631)Journal of Clinical Oncology,Vol 16,No 5,pp.172328,1998CSI:cranial-spinal junction site THE CRANIAL-SPINAL JUNCTION IN MEDULLOBLASTOMA:DOES IT MATTER?Int.J.R
9、adiation Oncology Biol.Phys.,Vol.44,No.1,pp.8184,1999Organ low junction(SD)high junction(SD)Cord 40.3Gy(0.5)38.4Gy(1.3)Thyroid gland 20.3Gy(9.2)26.3Gy(0.6)Mandible 6.2Gy(0.6)10.9Gy(5.1)Larynx 8.3Gy(3.9)27.2Gy(0.4)Pharynx 11.9Gy(5.1)20.3Gy(4.8)Parotid gland 14.9Gy(4.2)14.1Gy(4.2)超分割放疗Twice-daily l-Gy
10、 fractions were administered separated by 4-6 h.放疗剂量和射野同常规分割SRT Boost to posterior fossaPOSTERIOR FOSSA BOOST IN MEDULLOBLASTOMA:AN ANALYSIS OF DOSE TO SURROUNDING STRUCTURES USING 3-DIMENSIONAL(CONFORMAL)RADIOTHERAPY Int.J.Radiation Oncology Biol.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 Hypothyroid p值值年龄1 5岁 7/7(100%)10岁 2/10(20%)照射剂量123.4Gy+CT 10/12(83%)3岁、低危者(CSF-)、BED超过50Gy、放疗持续时间小于50天提示预后较好;低剂量CSI(23.4Gy)/低剂量CSI+化疗/HFRT有助于减轻低危患者远期并发症但并不能增加治愈率;术后应立即开始放疗,一般不推荐放疗前化疗;根据危险性分级选择放疗剂量和化疗方案。谢谢!谢谢!