髓母細(xì)胞瘤的放射治療

1、髓母細(xì)胞瘤的放射治療髓母細(xì)胞瘤的放射治療曹嫣娜概述概述 來源：胚胎殘留的未分化的原始髓樣上皮細(xì)胞。 部位：第四腦室頂上的小腦蚓部。 發(fā)病率：2.1/10萬/年，占兒童顱內(nèi)惡性腫瘤的1520%。 疾病特點(diǎn)：惡性程度高。 生長極其迅速； 手術(shù)難以完整切除； 腫瘤細(xì)胞易沿腦脊液播散（1646%）。臨床表現(xiàn)臨床表現(xiàn) 顱內(nèi)壓增高：頭痛、嘔吐、視神經(jīng)乳頭水腫 小腦損害：軀干性共濟(jì)失調(diào)為主 其它：復(fù)視、面癱、強(qiáng)迫頭位、頭顱增大、病理反射陽性、嗆咳、小腦危象、蛛網(wǎng)膜下腔出血 脊髓轉(zhuǎn)移灶癥狀：背部或雙下肢痛、進(jìn)行性加重的截癱或四肢癱分級分級Stage Risk staging system Stage Chan

2、gs M staging 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 fou

3、nd inGroup 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治療方案

4、 標(biāo)準(zhǔn)治療方案(“Philadelphia protocol”) 手術(shù) 放療：術(shù)后28天內(nèi)開始。 化療(VCP)：放療中VCR1.5mg/m2/w，共8周； 放療后6周開始CCNU75mg/m2 DDP75mg/m2 VCR1.5mg/m2/w3w， 每6周一個(gè)周期，共8個(gè)周期。放療劑量 低危組：CSI 23.4Gy/13f+后顱窩加量至 54Gy 高危組：CSI 36Gy/20f+后顱窩加量至54Gy放療技術(shù) 常規(guī)分割CSI+ Boost to posterior fossa 超分割CSI+ Boost to posterior fossa SRT Boost to posterior fo

5、ssaCraniospinal irradiation (CSI):methods 俯臥位，雙手置于體側(cè) 頭部兩側(cè)對穿野照射全腦及上段頸髓 單后野照射脊髓 各野皮膚間隔1cm 每照射10Gy移動一次射野以減少各野間交叉高劑量 6MV-X線照射 劑量(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

6、% 8% 75% 7% 69% 8% Prospective randomised trial 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的療效Risk-adapted cranio

7、spinal radiotherapy followed by high-dose chemotherapy 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 Medulloblasto

8、ma-96): long-term results from a prospective, multicentreMedulloblastoma-96): long-term results from a prospective, multicentre trial trial http:/oncology.the VolVol 7 October 2006 7 October 200623.4GyCSI對智力的影響(POG-8631)Journal of Clinical Oncology, Vol 16, No 5, pp. 172328, 1998CSI:cranial-spinal j

9、unction site THE CRANIAL-SPINAL JUNCTION IN MEDULLOBLASTOMA: DOES IT MATTER? Int. J. Radiation 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

10、.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) 超分割放療 Twice-daily l-Gy fractions were administered separated by 4-6 h. 放療劑量和射野同常規(guī)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 放療反應(yīng) 急性反應(yīng)：骨髓抑制、腦水腫等； 遠(yuǎn)期副作用： 甲低 認(rèn)知障礙 其它：聽力減退、骨骼發(fā)育障礙、周圍組織損傷繼發(fā)第二惡性腫瘤等。甲低 Hypothyroid p值值年齡1 5歲 7/7 ( 100% ) 10歲 2/10 ( 20%