综合治疗儿童髓母细胞瘤33例临床分析_郑伟

综合治疗儿童髓母细胞瘤33例临床分析

郑伟,聂青,康静波,杨平,张丽萍,李方明

[摘要] 目的 回顾性分析髓母细胞瘤的临床特点,探讨合理治疗方案,分析预后影响因素。方法 2004年8月-2007年12月共收治髓母细胞瘤术后患儿33例。术后3周内开始放疗,先予以全脑全脊髓28~36Gy,再予瘤床处三维适形局部补量至总量50~ 54Gy,常规分割剂量1 8~2 0Gy/次;为减轻晚反应,部分患儿采用超分割照射技术(1 0Gy/次、2次/d);所有患者在6~8周内完成放疗。全脑全脊髓放疗完成后行1个周期同步化疗,全部放疗结束后再进行序贯化疗3~5个周期;化疗方案为洛莫司汀+长春新碱+顺铂或异环磷酰胺+卡铂+依托泊苷。根据肿瘤是否转移、残存及残存肿瘤大小将患儿分为两组:低风险组(n=24):无转移、肿瘤全切或近全切(残留肿瘤体积 1 5cm3);高风险组(n=9):已有转移或残留肿瘤体积>1 5cm3。比较两组患者的3年生存率。结果 术后残留病灶患儿的放化疗有效率为90 9%(10/11),全组完全缓解(CR)93 9%(31/33),部分缓解(PR)3 0%(1/33),稳定(SD)3 0% (1/33)。所有患者中位生存时间51个月,3年总生存率和无病生存率分别为78 8%、75 8%。高风险和低风险患儿的3年生存率分别为33 3%和95 8%(P<0 01)。不良反应主要为血液系统和消化系统的毒性反应: - 度骨髓抑制发生率为21 2%(7/33),恶心发生率87 9%(29/33),伴呕吐者66 7%(22/33)。结论 儿童髓母细胞瘤的诊断不难,首选手术治疗,术后联合放化疗的总体疗效好,严重不良反应的发生率较低。肿瘤清除程度是影响预后的因素之一。

[关键词] 髓母细胞瘤;放射治疗剂量;预后

[中图分类号] R730 264 4 [文献标志码] A [文章编号] 0577 7402(2011)06 0657 04

C om bined radiother apy and chem ot herapy for pediatric m edu lloblastom a:a clinical study of33c ases

ZHENG Wei,NIE Qing,KANG Jing bo,YANG Ping,ZH ANG Li ping,LI Fang ming Department of Radiation Oncology,Naval General Hospital,Beijing100048,China

[A bstract] Objectiv e To retrospectively review the clinical characteristics of medulloblastoma,discuss the optimized treatment regimen,and analyze the prognostic influential factors.M ethods Thirty three children with pathologically certified medulloblastoma(aged 3-14years with average of6 5years),admitted from Aug.2004to Dec.2007,received radiotherapy within3weeks post surger y. Ratiotherapy consisted of28~36Gy whole craniospinal radiation and a supplementary radiation aimed at tumors by three dimensional conformal radiotherapy(3D CRT)for a total dose of50~54Gy(conventi onal fraction dose of1 8-2 0Gy).A part of patients received hyperfractionation radiotherapy(1 0Gy/f,2f/d)for alleviating the tardive adverse events.M eanwhile,a synchronized chemotherapy, consisting of lomustine+vincristine+cisplatin,or isophosphamide+carboplatin+eto poside,was administered after the completion of whole craniospinal radiation,and3-5courses of sequential chemotherapy were given after the overall radiotherapy was fi nished.According to the metastasis,and the residual tumor and its size,the33patients were divided i nto2groups as fo llows:low risk g roup(n=24):no metastases,total or sub total excision of tumors(residual tumors 1 5cm3);high risk group(n=9):ei ther metastases or residual tumor >1 5cm3.T he3 year survival rates of two groups were then compared.Results T he combined radi otherapy and chemotherapy was effective to10of the11patients(90 9%)with residual tumors.Out of the33patients,31obtained complete remission(93 9%),and2 patients showed partial remission or stable status(3 0%,respectively).T he median survival time of33patients was51months,3 year disease free survival(DFS)was75 8%,and3 year overall survival(OS)was78 8%,including33 3%in high risk g roup and95 8%in low risk group(P<0 01).The major side effects occurred in haematological system and digestive system,such as an incidence of21 2% (7/33)with grade - bone marrow suppression,87 9%(29/33)with nausea,and66 7%(22/33)with nausea and vomiting.

C o nclusions It is not difficult to make a definite diagnosis of pediatric bined radiotherapy and chemotherapy following surgery,which is primarily recommended therapy,will give favorable effects with a low risk of complicating severe adverse events.T he eradication of the tumor plays a pivotal role in influencing the prognosi s of patients.

[Key words] medulloblastoma;radiotherapy dosage;anti neoplastic combined chemotherapy protocols;prognosis

髓母细胞瘤(medulloblastoma,MB)是中枢神经系统高度恶性的神经上皮性肿瘤,约占儿童原发性颅内肿瘤的20%,其发病率仅次于星形细胞瘤[1]。MB好发于10岁以下儿童,患儿年龄5~7岁[2],男性多于女

[作者简介] 郑伟,医学学士,主治医师。主要从事临床常见肿瘤的放化疗研究

[作者单位] 100048 北京 海军总医院放射肿瘤科(郑伟、聂青、康静波、杨平、张丽萍、李方明)性。该肿瘤可发生于脑组织任何部位,绝大多数生长在第四脑室顶的小脑蚓部,易造成脑脊液循环梗阻而引发颅内高压,但小儿颅内高压症状不甚典型,故易误诊。髓母细胞瘤的高度恶性主要表现在3个方面:生长极其迅速,手术不易全部切除,肿瘤有沿脑脊液产生播散种植的倾向。所以,采用单一手段治疗髓母细胞瘤效果往往不理想,目前公认的治疗原则是采用术后联合放化疗进行综合治疗。本研究收集海军总医院放