86例髓母细胞瘤患儿的随访和临床分析

目的旨在探索性别、部位、免疫组化指标、切除程度、是否行术后放疗和化疗等临床病理因素对髓母细胞瘤预后的影响。方法对86例髓母细胞瘤患儿进行回顾性队列研究,收集患儿临床资料信息,并进行电话随访。使用Kaplan-Meier曲线和Cox多因素回归进行生存分析,寻找影响髓母细胞瘤预后的独立危险因素。结果 86例患儿中,男52例(60.5%),女34例(39.5%),平均年龄为(10.9±3.8)岁。肿瘤部位:小脑内46例(53.5%),突入四脑室40例(46.5%)。59例(68.6%)实现了肿瘤全切。接受术后放疗70例(81.4%),术后放疗的基础上行化疗44例(51.2%)。队列5年无进展生存率为45.8%,整体生存率为(50.4%)。多因素分析:年龄10.5岁(P=0.021)、确诊时无转移(P=0.045)、术后放疗(P0.001)和突触素表达≥50%(P=0.007)与较好的无进展生存期相关。术后行放疗的基础上再行化疗,对疗效并无贡献(P=0.262)。结论在髓母细胞瘤患儿中,术后行放疗是必需的,术后放疗后再行化疗尚存争议。年龄、转移、术后放疗和突触素表达被证实为髓母细胞瘤患儿预后的独立危险因素。免疫组化指标在髓母细胞瘤具有预后预测价值。     

髓母细胞瘤的治疗与预后分析

目的探讨儿童及成人髓母细胞瘤患者的治疗及预后情况。方法回顾性研究80例经病理证实的髓母细胞瘤，对其成人与儿童患者的生存情况与肿瘤切除程度、脑脊液分流情况、放疗方式对其预后的影响作多因素分析比较。结果61例（76％）获得6个月至14年随访。5年生存率与10年生存率分别为50．8％和27．9％。成人患者5年生存率较儿童高（P＜0．05），而10年生存率两组相近。肿瘤全切或次全切除生存率高于大部分切除（P＜0．05）。全脑加脊髓放疗可改善患者近期生存率（P＜0．05）。3例患儿生存超过Collins危险期。结论手术与术后常规放疗是治疗髓母细胞瘤的关键。全切除或次全切除肿瘤、全脑加脊髓放疗可明显提高生存率。少数患者有可能获得长期生存。     

成人髓母细胞瘤的诊断和治疗

目的 探讨成人髓母细胞瘤的临床特点、诊断和治疗方法.方法 回顾性分析我院2000 年8月至2008 年12 月期间收治的16 例成人髓母细胞瘤的临床及影像学表现、治疗措施与效果.结果 肿瘤全切除12 例,次全切除3 例,部分切除1 例,术后均行放疗,3 例同时行化疗.术后复发3 例,术后2 年生存率86.7%,5 年生存率66.7%.结论 成人髓母细胞瘤发生于后颅窝中线区者多见.手术切除及术后全脑脊髓放疗是主要治疗方法.复发者预后差.     

儿童颅后凹髓母细胞瘤的诊断及手术治疗

目的分析儿童髓母细胞瘤的临床特点及手术治疗方法与疗效。方法总结47例经手术病理证实的儿童髓母细胞瘤病人的临床资料。肿瘤均位于颅后凹,其中小脑蚓部36例,小脑半球11例。术后均予放疗与化疗。结果肿瘤全切除29例,大部切除16例,部分切除2例。死亡3例。随访2~10年,肿瘤全切者生存率63.4%,未全切者生存率23.7%;平均生存26.7个月。结论手术全切除肿瘤,解除导水管梗阻,术后予全脑加全脊髓放疗,可延长生存期。     

成人髓母细胞瘤的临床特点与治疗

目的研究成人髓母细胞瘤的临床特点及治疗措施。方法20例成人髓母细胞瘤，其中男性13例，女性7例，平均年龄26．6岁，平均病程5．5月。所有病人接受显微神经外科手术治疗，15例病人术后接受放疗，4例行术后化疗。结果肿瘤全切除15例，近全切除2例，部分切除3例，无手术死亡。术后复发6例，中枢神经系统种植转移2例，术后2年生存率90％，术后5年生存率75％。结论通过手术全切除肿瘤和术后全中枢轴放疗可使成人髓母细胞瘤患者得到较好的治疗效果。     

成人髓母细胞瘤患者脑(脊)膜播散种植的诊断与治疗

目的探讨成人髓母细胞瘤患者发生脑(脊)膜播散种植后的临床诊断与治疗策略。方法回顾分析11例成人髓母细胞瘤患者发生脑(脊)膜播散种植后的临床表现、脑脊液细胞学特点、影像学表现和治疗措施。结果 11例患者中10例采用枕下后正中入路、1例采用乙状窦后入路全切除肿瘤,恢复脑脊液循环通路。7例患者术后放射治疗前复查MRI即发现脑(脊)膜播散种植,予以放射治疗和药物化疗等综合治疗后好转;4例术后复查MRI未见脑(脊)膜播散种植,行常规放射治疗,随访3个月至3年(平均20个月),复查MRI显示脑(脊)膜播散种植。仅2例患者术前脑脊液细胞学检查发现肿瘤细胞。所有患者均行放射治疗和药物化疗,生存2例、死亡9例。结论成人髓母细胞瘤发生脑(脊)膜播散种植者预后较差,MRI较脑脊液细胞学更为敏感,予放射治疗联合药物化疗的综合治疗措施可以延长生存期、提高生活质量。     

27例儿童胶质母细胞瘤临床治疗和预后相关因素分析

目的探讨儿童胶质母细胞瘤的治疗效果及预后相关因素。方法回顾性分析2010年至2017年间收治的27例18岁以下儿童胶质母细胞瘤的手术、放疗与化疗情况,进行长期追踪随访,并对性别、年龄、手术切除程度,以及放化疗4个预后相关因素进行探讨。结果所有患儿均接受了开颅手术治疗。包括21名男性和6名女性。24例肿瘤位于幕上,其余3例位于幕下。22例肿瘤得到全切除,另外5例得到次全切除,围手术期无病人昏迷和死亡。8例患儿在手术后接受了放疗和化疗,9例接受了单纯的化疗,另外10例未进行任何放化疗。本组病例中位生存时间16个月,6个月总生存率81.5%,1年总生存率59.3%,2年总生存率33.3%,5年总生存率14.8%。男性患儿中位生存时间13个月,女性中位生存时间14个月,在性别上无统计学上的显著差异(P=0.87)。0~10岁组患者中位生存时间11个月,11~18岁组患者中位生存时间14个月,在年龄上无统计学显著差异(P=0.63)。单纯手术组中位生存时间8.5个月,而手术+放化疗组中位生存时间为16个月,两组在预后上有显著差异(P=0.0067)。肿瘤全切组中位生存时间为14个月,而次全切除组中位时间为12个月,虽然无明显的统计学差异(P=0.14),但是获得长期生存的病例均来自肿瘤全切患儿。结论儿童胶质母细胞瘤的总体预后较差,生存时间与性别和年龄无关,术后放化疗对延长生存期具有重要的意义。全切肿瘤不能明显延长患儿生存时间,但是患儿获得长期生存的重要前提。     

颅内横纹肌样脑膜瘤三例并文献复习

横纹肌样脑膜瘤是一种罕见的颅内肿瘤,恶性程度高,易于复发、转移,临床预后差。本文报道3例横纹肌样脑膜瘤患者,结合苏木素-伊红染色及免疫组织化学染色诊断明确。其中1例术后1个月死于大面积脑梗死,1例术后行放疗和(或)化疗后2年死于胸腔积液,1例术后至今仍存活(术后生存时间>25个月)。横纹肌样脑膜瘤的诊断需结合组织病理学特征和免疫组织化学染色确诊,术后行影像学定期随访十分必要,对手术未完全切除肿瘤的患者建议术后辅助放射治疗。     

成人幕上原始神经外胚层肿瘤的诊治分析(附10例报道)

目的探讨成人幕上原始神经外胚层肿瘤(PNET)的临床特点、诊断及治疗方法。方法回顾性分析2011年11月至2015年12月收治的10例成人幕上PNET的临床资料,均行手术治疗,术后均行辅助放疗,辅助化疗5例。结果肿瘤全切除1例,近全切除6例,大部分切除3例。术后随访3~48个月;5例死亡。5例术后辅助单纯放疗,随访期间死亡3例,死亡时间为术后2、6、8个月;术后辅助放、化疗5例,随访期间死亡2例,死亡时间为术后12、22个月。1例额叶PNET,手术全切肿瘤,术后辅助放、化疗,随访期间未见复发。结论成人幕上PNET为颅内罕见肿瘤,恶性度高,易沿脑脊液播散,预后差;确诊依赖病理检查;采用手术、放疗、化疗、免疫治疗等综合治疗有助于延长患者生存期。     

成人颅内神经母细胞瘤(1例报告并文献复习)

目的探讨原发性颅内神经母细胞瘤的临床、影像及病理学特点,以及治疗策略和预后。方法回顾性分析南京军区福州总医院神经外科收治的1例成人左侧额叶神经母细胞瘤患者的临床资料,将其临床、影像及病理学特点结合相关文献进行分析,并对患者进行跟踪随访。结果本例患者行神经导航下开颅肿瘤切除术,病理学检查证实为颅内神经母细胞瘤,术后以替莫唑胺同步放、化疗,后继以6周期辅助化疗。患者已无瘤生存4年,无明显神经功能缺失表现。结论成人颅内神经母细胞瘤极为少见且恶性程度高,手术完全切除肿瘤及术后正规放、化疗可显著提高患者的生存率。     

173例胶质瘤预后的影响因素分析

目的 探讨影响胶质瘤预后的相关因素。方法 对2000年1月至2009年12月在中山大学肿瘤防治中心首次手术病理确诊为胶质瘤的临床资料进行回顾性分析,并排除因非肿瘤因素死亡患者,采用Kaplan-Meier法进行生存率估计及Cox比例风险回归模型进行预后多因素分析。结果 本组纳入胶质瘤173例,其中WHO Ⅰ级10例,Ⅱ级61例,Ⅲ级53例,Ⅳ级49例;98例术后接受放疗[高级别胶质瘤（WHOⅢ~Ⅳ级）61例,低级别胶质瘤（WHO Ⅰ~Ⅱ级）37例];60例术后接受化疗（高级别胶质瘤46例,低级别胶质瘤14例）。本组患者1、3、5年总体生存率分别为74.0%、42.2%、32.4%;WHO Ⅰ、Ⅱ、Ⅲ、Ⅳ级的 5年生存率分别为80.0%、52.5%、24.5%、6.1%。分层分析显示术后辅助放化疗显著影响高级别胶质瘤生存率（P <0.05）。Cox比例风险回归模型分析结果 显示,年龄>40岁（RR=1.603;P=0.019）、WHO Ⅲ~Ⅳ分级（RR=2.311;P <0.001）、肿瘤未全切（RR=2.108;P <0.001）、术后未放疗（RR=1.652;P=0.008）是影响胶质瘤总体生存率的独立危险因素。结论 本组病例的分析结果 提示,发病年龄≤40岁、WHO级别低、肿瘤全切的胶质瘤患者预后好;术后进行放疗可以提高胶质瘤的疗效。     

Efficacy of adjuvant chemotherapy for patients with poor-risk medulloblastoma: a preliminary report

Recent studies have demonstrated that preoperative and postoperative factors can broadly stratify patients with medulloblastoma/primitive neuroectodermal tumors (MB/PNET) into risk groups. For children with factors that suggest poor outcome after treatment with surgery and radiotherapy, the addition of chemotherapy can improve survival. Since 1983, 26 children with poor-risk posterior fossa MB/PNET have been treated at our institution with craniospinal radiation therapy and adjuvant chemotherapy. Chemotherapy consisted of vincristine during radiotherapy and eight 6-week cycles of vincristine, cis-platinum, and 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU). Twenty-five of 26 patients (96%) who have been entered on this protocol remain alive and free of disease at a median of 24 months from diagnosis (range 6 to 50 months). Twenty patients have completed all therapy and are at a median of 32 months from initial diagnosis with no evidence of disease. These patients were compared to a group of children with similar prognostic features treated at our institution between 1975 and 1983. Actuarial disease-free survival was statistically significantly better for protocol patients than for historical control subjects (p less than 0.002). This difference was most marked in patients who had received radiation therapy alone (p less than 0.0003). Actuarial 2-year disease-free survival was 96% for patients on protocol as compared to 59% for historical control patients who had been treated with radiotherapy alone. The chemotherapy given in this protocol was well tolerated. The results of this study, although preliminary, suggest that adjuvant chemotherapy is at least transiently effective in improving the rate of disease-free survival for children with poor-risk MB/PNET.     

Use of adjuvant ICE chemotherapy in the treatment of anaplastic ependymomas

Despite the improved outcome for patients with ependymoma treated by surgery and radiotherapy, their prognosis remains relatively poor. To assess the impact of adjuvant chemotherapy, we reviewed the overall survival of consecutive patients with anaplastic ependymoma treated over a 10-year period with surgery and ICE (ifosfamide+ VP16+carboplatin) chemotherapy with or without radiation at our institution. There were 11 patients (6 male, 5 female), with a median age of 3.4 years (range 1.2–11.1): 4 under 2 years and 7 were over 2 years old. Overall, 5 had gross total resections: 4 are alive, 2 in continuous complete remission and 2 in second complete remission. One patient who was less than 2 years old died. Among the 6 with subtotal resection, 2 achieved a complete remission after chemotherapy. However, 5 of the 6 patients have since died of progressive disease, with a median overall survival of 75 months. Overall survival was 24% and progression-free survival was 39%. In 2 of 6 patients with residual postoperative disease a temporary objective response was noted with adjuvant ICE chemotherapy. This regimen did not confer an overall survival advantage. Received: 24 July 1998     

92例高级别胶质瘤患者术后生存分析

目的探讨影响高级别胶质瘤术后生存的因素。方法对2014年1月至2016年8月首诊确定为高级别胶质瘤(WHO III-IV级)的92例患者采用Kaplan-Meier法分析生存率,Log-rank检验进行单因素分析,Cox回归模型进行多因素分析。结果中位生存期为15个月,中位无进展生存期为8个月,肿瘤全切率为72. 83%,1年、2年、3年、4年的生存率分别为56. 5%,35. 9%,30. 4%,25. 4%。Log-rank单因素分析表明高级别胶质瘤预后与年龄、术前生活质量评分、肿瘤大小、数目、是否累及多个皮层脑叶或者运动功能区、切除程度、病理级别、异柠檬酸脱氢酶基因是否突变、O-6-甲基鸟嘌呤DNA甲基转移酶基因启动子是否甲基化、Ki 67指数、术后是否进行放疗和/或化疗以及所进行的放化疗的方式、复发后是否积极治疗相关(P 0. 05)。引入检验水准(α=0. 01),COX多因素分析表明年龄、肿瘤切除程度、术后是否进行放疗和/或化疗是影响高级别胶质瘤预后的独立危险因素(P 0. 01)。结论发病年龄65岁、肿瘤全切除、术后进行同步放化疗和辅助化疗的高级别胶质瘤患者预后较好。     

Efficacy of temozolomide as adjuvant chemotherapy after postsurgical radiotherapy alone for glioblastomas

Objectives

The aim of this study was to assess the efficacy of adjuvant TMZ chemotherapy for newly diagnosed GBM patients who were treated with surgery followed by radiotherapy alone.

Material and methods

Between January 2003 and April 2005, 59 consecutive GBM patients underwent radiation therapy after surgical resection and subsequently received TMZ chemotherapy. For the comparative analysis, we selected 60 clinically matched GBM patients who underwent radiotherapy followed by nitrosourea-based chemotherapy (NUBC), at the same institution between June 1995 and April 2005. The study cohort was divided into two groups, those with adjuvant TMZ treatment and with NUBC.

Results

59 patients with adjuvant TMZ treatment were assigned to the treatment group and 60 patients with NUBC to the control group. The median overall survival for the treatment group was 18.2 months (95% CI, 11.7–24.7 months), compared with the survival of 14.5 months (95% CI, 11.2–17.7 months) for the control group (p = 0.019). The progression-free survival for the treatment group was 5.6 months (95% CI, 4.4–6.7 months), while the control group showed progression-free survival of 3.3 months (95% CT, 3.2–6.0 months) (p = 0.030). Uni- and multivariate analysis revealed that extent of surgical resection, age ≥55 years and postoperative KPS were significantly associated with survival.

Conclusion

Adjuvant TMZ chemotherapy provided a clinically relevant benefit of survival, as compared with NUBC. Thus, we suggest that adjuvant TMZ chemotherapy may be effective even for patients who did not receive concomitant chemoradiotherapy for GBM.     

儿童髓母细胞瘤患者临床因素与预后的相关性分析

目的 探讨影响儿童髓母细胞瘤(MB)患者预后的临床因素.方法 选择中山大学附属第一医院神经外科自2001年11月至2010年7月收治的儿童MB患者47例,采用Kaplan-Meier生存分析法和Cox回归模型分析患者年龄、性别、病程、肿瘤部位、术前转移、肿瘤切除程度、病理亚型、脑干侵袭程度、放疗与手术的时间间隔、化疗对生存时间即预后的影响.结果 本组患者2年生存率为91.4%,5年生存率为50%.Kaplan-Meier生存分析显示肿瘤全切或次全切者生存率高于大部分切除者,术前无转移患者生存率高于有转移者,放疗与手术的时间间隔≤42 d者生存率高于时间间隔>42 d者,差异均有统计学意义(P<0.05).Cox 回归模型分析显示术前转移、放疗与手术的时间间隔为儿童MB患者预后的影响因素,术前无转移、放疗与手术的时间间隔≤42 d患者预后较好.结论 术前有无转移和放疗与手术的时间间隔是影响儿童MB患者预后的独立因素.

Abstract：

Objective To analyze the correlation between the prognosis of pediatric medulloblastoma (MB) and its relevant clinical factors. Methods Forty-seven children with MB,admitted to our hospital from November 2001 to July 2010, were chosen;a retrospective analysis, using Kaplan-Meier survival tested by log-rank and Cox proportional hazards regression model, was performed to identify the correlation between the prognosis of pediatric MB and relevant clinical factors which included age, gender, course of disease, tumor location, pathological subtype, preoperative metastasis,extent of tumor resection, interval between surgery and radiation, brainstem invasion and chemotherapy.Results The 2-year survival rate of these patients was 91.4%, and 5-year survival rate 50%.Kaplan-Meier curves indicated that patients with total or subtotal tumor resection had a significantly higher survival rate than those with partial resection (P<0.05);patients without preoperative metastasis had a significantly higher survival rate than those with preoperative metastasis (P<0.05);and patients with interval between surgery and radiation≤42 d had a significantly higher survival rate than those with interval between surgery and radiation >42 d (P<0.05). Cox proportional hazards regression analysis indicated that preoperative metastasis (RR, 6.808;95%CI, 1.844 to 25.113;P<0.05) and interval between surgery and radiation (RR, 5.642;95% CI, 2.042 to 15.587;P<0.05) were the influencing factors for prognosis of MB. Patients without preoperative metastasis and with interval between surgery and radiation ≤42 d enjoyed good prognosis results. Conclusion Both preoperative metastasis and interval between surgery and radiation are independent factors for prognosis of pediatric MB.     

原发性人脑胶质瘤的预后影响因素:基于SEER数据库的分析

目的 探讨原发性人脑胶质瘤病人预后影响因素。方法 通过SEER*Stat（8.3.8版本）软件搜集SEER数据库2004~2015年原发性人脑胶质瘤病人的临床资料,采用R（4.0.2版本）软件进行单因素与多因素Cox回归分析,通过Kaplan-Meier生存曲线分析不同治疗方式和婚姻状态与病人预后的关系。结果 共纳入符合标准的原发性人脑胶质瘤18 523例。多因素Cox回归分析结果显示,年龄≥30岁、肿瘤直径≥2 cm、病理级别高、肿瘤位于额叶以外部位、离婚、丧偶为原发性胶质瘤预后不良的独立危险因素（P<0.05）,手术治疗、术后放疗、化疗是原发性胶质瘤预后的独立保护因素（P<0.05）。生存曲线分析结果显示,手术治疗（全切除或部分切除肿瘤）、术后放疗、化疗均明显延长原发性胶质瘤病人的生存期（P<0.05）,婚姻状态为结婚的病人预后明显好于丧偶的病人（P<0.05）。结论 对原发性人脑胶质瘤,尽可能手术全切除肿瘤,同时术后辅助放化疗,能够延长病人的生存时间。同时,临床应加强病人心理干预。     

Diffuse and focal brain stem tumors in childhood: prognostic factors and surgical outcome

Objective Brainstem tumors (BSTs) are usually gliomas and are divided into diffuse BSTs (DBSTs) and focal BSTs (FBSTs). The aim of this study is to investigate the different outcomes of these two entities.Methods Thirty-one patients with BSTs were admitted to our institution from 1995 to 2003. Patients with DBSTs were treated with locoregional radiotherapy (1.8 Gy/day for 54 Gy) and weekly vincristine for radiosensitization (1.5 mg/sm for six total doses). Patients with FBSTs underwent surgical resection. Chemotherapy and/or radiotherapy were considered in progression.Results and conclusions Fourteen patients were diagnosed as having DBSTs. The responses to treatment were ten cases of partial response, three of stable disease, and one of progressive disease. General and/or neurological symptoms improved in more than 80% of patients. The median time from diagnosis to progression and to death were, nonetheless, 8 (range of 3–13) and 13 (range of 4–25) months, respectively, with a 2-year overall survival rate of 12.3% [standard error (SE) 11.2]. Seventeen patients were diagnosed as having FBSTs. Gross total removal was achieved in 4/17 cases, subtotal removal in 7/17, and partial removal in 6/17. There was one surgery-related death. Eight out of 17 patients had adjuvant chemo- and/or radiotherapy after progression: 6/8 are without neurological symptoms and 2/8 have died due to tumor progression. The 4-year overall and disease-free survival rates are 87.4 (SE 8.4) and 58.8% (SE 11.9), respectively, the extent of resection being the most important prognostic factor (p=0.012). DBSTs continue to carry a dismal prognosis, thus demanding new treatment modalities; FBSTs can be treated surgically and patients benefit from a better prognosis.     

术前加术后中性粒细胞与淋巴细胞比值预测胶质瘤患者的预后研究

目的 研究术前加术后外周血中性粒细胞与淋巴细胞比值(NLR)对胶质瘤患者预后的预测价值.方法 回顾性分析110例经手术、病理检查确诊的胶质瘤患者的临床资料.将术前和术后NLR低于临界值的记为0,术前和术后NLR高于临界值的记为1.术前NLR加术后NLR记为PP-NLR,则PP-NLR=0或1或2;按患者的PP-NLR将...