小脑髓母细胞瘤术后放射治疗—44例疗效观察

本文报导44例髓母细胞瘤术后放疗的疗效,其一、三、五年生存率分别为81.82%,31.82%及(?)6.67%,与国内报告相仿.分析了治愈率较低的原,并提出改进治疗的意见.     

Medulloblastoma in childhood: progressive intellectual deterioration

A series of 120 medulloblastomas in children operated on between 1967 and 1987 at the Hôpital des Enfants-Malades has been reviewed in order to check whether the conclusions of our study published 10 years ago have remained valid and, in particular, to verify whether the quality of life of these patients, which had been found to be poor at the time, had improved or worsened over the years. The postoperative mortality for the whole series was 6.5% there have been no deaths in the 35 patients operated on after 1980. The overall survival rate for the 120 children was 60% at 5 years and 53% at 10 years; for the patients who completed radiotherapy, the survival rates was 73% at 5 years and 64% at 10 years. Survival rates were surprisingly better in patients treated when under 6 years of age than in older children. They were also better in girls than in boys, and in desmoplastic compared with other medulloblastomas; however, the differences were not significant. When comparing the groups after total or subtotal resection of tumors, survival rates were not significantly different, but were lower in the small group of partial resections. Cell differentiation did not influence the prognosis. Psychological sequelae were significant and worsened over the years. Five years after treatment 58% of the children showed an IQ above 80; 5 years later this group included only 15% of the patients. These psychological sequelae were related to age at the time of radiotherapy: the younger the child, the lower the final IQ. Five years after treatment, 40% of the children had a normal academic level; 5 years later this group was reduced to 11%. Ten years after treatment, 36% of the patients were unempolyed and 64% worked in a protected environment. No patient had normal employment. These disastrous results concerning the quality of life of these medulloblastoma patients justify new trials with reduced doses of irradiation over the hemispheres, at least in low-risk groups.     

EGFR、PCNA、N-myc和P16基因蛋白产物在髓母细胞瘤中的表达

目的探讨EGFR、Nmyc、PCNA和P16基因在髓母细胞瘤发生中的作用,以及上述基因蛋白检测对髓母细胞瘤的诊断、病理分型和年龄的临床意义。方法用免疫组织化学LSAB法检测四种基因蛋白的产物在41例人脑髓母细胞瘤中的表达,分析与髓母细胞瘤病理分型和年龄间的关系。结果EGFR、Nmyc和PCNA蛋白在髓母细胞瘤中高度表达,P16蛋白有明显的缺失,分别为56.10%、60.98%、65.89%、58.54%。EGRF蛋白在两种主要病理类型中的表达差异有统计学意义(P<0.01),P16蛋白在两组不同年龄组间的差异有统计学意义(P<0.05)。结论上述四种基因在髓母细胞瘤的发生中可能起重要作用,EGFR和P16蛋白的检测对病理分型和预后判断有临床实际应用意义。     

髓母细胞瘤91例的围手术期护理

本文就１９８７年～１９９６年华西医科大学附一院神经外科和长治医学院附院神经外科收治并经手术和病理证实９１例髓母细胞瘤的围手术期护理进行回顾性总结。针对临床表现以颅内压增高和小脑共济失调为主，肿瘤易侵犯脑干的特点，作者强调术前应严密观察病情变化，充分的术前准备，包括良好的心理护理和生活护理，以有利于手术的顺利进行。术后除常规观察外，还应帮助采取正确的卧位，强调呼吸功能监护的重要性及其它临床监护，确保     

不同联合放化疗方式对儿童髓母细胞瘤疗效的系统评价

目的系统评价不同的联合放化疗方式对儿童髓母细胞瘤的疗效。方法计算机检索MEDLINE/Pub Med、Embase和Cochrane Library等数据库,对纳入的随机对照试验(randomized controlled trials,RCT)采用Rev Man5.3进行Meta分析。结果共纳入10篇RCT文献,1807例患者。Meta分析结果显示髓母细胞瘤术后联合放化疗与术后单纯放疗相比,能提高3年总生存率(overall survival,OS)[RR=1.18,95%CI(1.02,1.37),P0.05]和高危险组患者5年无事件生存率(event-free survival,EFS)或无病生存率(disease-free survival,DFS)[RR=1.27,95%CI(1.01,1.59),P0.05]。先放疗后持续化疗的方案和先化疗后放疗的"三明治"方案相比,能提高髓母细胞瘤M0级别患者5年OS[RR=1.46,95%CI(1.19,1.79),P0.05]和5年EFS/DFS[RR=1.29,95%CI(1.06,1.55),P0.05],能提高M1级别患者5年OS[RR=2.26,95%CI(1.03,4.97),P0.05]。结论手术切除后联合放化疗治疗儿童髓母细胞瘤效果优于术后单纯放疗。术后先放疗后化疗的方案优于术后先化疗后放疗的"三明治"方案。     

Characterization of anaplastic lymphoma kinase-positive medulloblastomas

Medulloblastomas are the most common pediatric malignant primary brain tumor. To our knowledge, there are no known critical and druggable tyrosine kinases in medulloblastomas, precluding the use of established tyrosine kinase inhibitors that have shown efficacy in other tumor types. We studied the expression of anaplastic lymphoma kinase (ALK), a well-characterized tyrosine kinase and drug target, in a cohort of medulloblastomas by immunohistochemistry, and identified three ALK-positive cases. Mutational analyses did not reveal a definite underlying genetic mechanism for the ALK expression, although one of the cases showed increased ALK copy number. Our findings have clinical implications and warrant further pharmacological and functional studies, as well as evaluation in larger patient cohorts, to fully characterize the value of ALK as a prognostic and predictive therapeutic marker in medulloblastomas.     

小儿髓母细胞瘤与室管膜瘤的术前误诊分析

儿童颅内肿瘤好发于中线部位和后颅凹,早期即可压迫脑室系统,影响脑脊液循环而致颅内压增高,压迫脑干等主要结构,故病情重,病程短。后颅窝肿瘤由于有颅底颅骨伪影干扰,CT影像又仅限于横轴位扫描,往往造成后颅窝肿瘤的鉴别诊断困难,而造成术前误诊[1]。本文回顾分析一组儿童后颅     

儿童髓母细胞瘤亚型的MRI特征

目的探讨分析儿童髓母细胞瘤组织病理学各亚型的MRI表现以及扩散加权成像(DWI)、氢质子磁共振波谱(1H-MRS)的诊断价值。方法回顾性分析经手术病理证实的136例儿童髓母细胞瘤患者,本组中所有患者均在术前行MRI、DWI检查,其中50例同时行1H-MRS检查,比较分析各亚型间的平均ADC值(ADC_(mean))、最小ADC值(ADC_(min))、r ADC及代谢物的差异性。结果 136例中,经典型83例,促结缔组织增生/结节型28例,髓母细胞瘤伴广泛结节型11例,大细胞/间变型14例。常规MRI征象(包括T_1WI平扫和T_2WI平扫表现、强化程度、瘤周水肿、囊变、出血改变、播散等)未见明显差异(P均0.05)。促结缔组织增生/结节型的ADC_(mean)、ADC_(min)、r ADC低于经典型、大细胞/间变型,且大细胞/间变型的ADC_(mean)、ADC_(min)、rADC在各亚型中最高(P均0.05)。髓母细胞瘤伴广泛结节型与经典型、髓母细胞瘤伴广泛结节型与促结缔组织增生/结节型之间的ADC_(mean)、ADC_(min)、r ADC差异无统计学意义(P均0.05)。各亚型的ADC_(mean)、ADC_(min)、r ADC大小为:大细胞/间变型经典型促结缔组织增生/结节型;而髓母细胞瘤伴广泛结节型与经典型、髓母细胞瘤伴广泛结节型与促结缔组织增生/结节型之间无明显差异。1H-MRS显示各亚型的Cho/Cr、NAA/Cr、NAA/Cho、m I/Cr以及Lip出现概率无统计学差异(P均0.05);但Lac出现概率差异具有统计学意义(P0.05),各亚型出现Lac峰的概率为:大细胞/间变型髓母细胞瘤伴广泛结节型经典型促结缔组织增生/结节型。结论儿童髓母细胞瘤各亚型的常规MRI表现、各代谢物比值及Lip峰出现概率无明显差异,但各亚型的ADC_(mean)、ADC_(min)、r ADC值及Lac峰出现情况有一定特征性,其与病理特征密切相关,在髓母细胞瘤各组织学分型的鉴别诊断上具有较高的价值。     

特异性抑制髓母细胞瘤1型胰岛素生长因子受体信号通路后的差异性基因表达

目的 应用聚合酶链反应(PCR)芯片技术观察特异性抑制髓母细胞瘤细胞1型胰岛素生长因子受体(IGF-1R)信号通路的差异性基因表达,探讨IGF-1R在髓母细胞瘤细胞增殖中的作用.方法 体外培养髓母细胞瘤Doay细胞株,使用不同终质量浓度NVP-ADW742作用48 h和24 h后,检测细胞增殖和凋亡,分别使用IGF-1R酪氨酸蛋白激酶抑制剂NVP-ADW742终质量浓度为0、0.5、1.0、2.0、5.0、10.0、20.0、50.0μmol/L作用48 h后检测细胞存活率,以及终质量浓度为0.1、1.0、2.0、5.0、10.0μmol/L作用24 h后检测凋亡率,应用PCR芯片技术检测IGF-1R信号通路的差异性基因表达,并采用Western blot检测其蛋白表达.结果 在对应浓度的NVP-ADW742作用下Doay细胞的存活率分别为95.95%、93.95%、91.97%、86.60%、70.57%、15.01%、1.63%(P＜0.05),凋亡率分别为6.92%、8.83%、11.22%、17.27%、29.98%(P＜0.05),两者呈剂量依赖性,其浓度越高,抑制和促凋亡作用越强;在PCR芯片检测有14个差异表达基因;Western blot检测表明在2μmol/L的NVP-ADW742作用下,随时间延长PI3K、Akt、p38、GSK-3β和bcl-2蛋白表达逐渐下调.结论 髓母细胞瘤中IGF-1R信号通路异常可以促进肿瘤细胞的增殖,利用功能性PCR芯片可以有效地检测肿瘤细胞中相关基因在肿瘤增殖和凋亡中的作用.     

小脑髓母细胞瘤30例临床病理分析

目的探讨髓母细胞瘤的临床病理特征。方法回顾性分析30例髓母细胞瘤的临床资料、光镜下特征和免疫组织化学染色的特点。结果患者30例,男16例,女14例;发病年龄5个月～40岁。临床表现为头痛头晕伴行走不稳,CT及MRI示后颅凹占位。镜下见肿瘤由未分化小圆细胞组成,呈弥漫性及结节状分布,可伴有少量间质。免疫组化染色神经元特异性烯醇化酶（NSE）、胶质纤维酸性蛋白（GFAP）、S-100蛋白阳性数分别为26/30、17/30、13/30。结论髓母细胞瘤为侵袭性强的恶性肿瘤,诊断应与非典型畸胎样/横纹肌样瘤、室管膜瘤、毛细胞性星形细胞瘤、淋巴瘤、小细胞未分化癌、胚胎型横纹肌肉瘤相鉴别。