显微手术治疗儿童第四脑室肿瘤

目的:对儿童第四脑室肿瘤的早期诊断、手术策略和技巧及术后并发症进行分析讨论.方法:回顾性分析中国医大盛京医院1993年1月至2008年12月儿童第四脑室肿瘤手术病例57例.所有患儿均经显微手术治疗.男33例,女24例.年龄9个月至14岁.所有患儿术前、术后均行头颅CT和(或)MRI检查.结果:本组手术全切除34例,次全切除20例,大部切除3例.症状较术前明显改善42例,无明显改善或加重5例;10例术后7d-2个月内行侧脑室-腹腔分流术.46例患者获得随访3-40个月,32例生活或学习良好,其中随访期内复发7例.对3岁以上的20例髓母细胞瘤,6例室管膜瘤切除术后行小剂量X线全脑脊髓神经轴放疗,6例星形胶质细胞瘤切除术后加化疗.结论:儿童第四脑室肿瘤以髓母细胞瘤最多见,其次为室管膜瘤和星形细胞瘤.早期正确诊断,术中恰当的手术策略选择及切除程度、术后并发症恰当处理和有选择性放疗是影响儿童第四脑室肿瘤预后的关键因素.     

第四脑室肿瘤的诊断和显微手术治疗

目的分析第四脑室肿瘤的诊断,探讨显微手术切除颅脑肿瘤的效果。方法86例颅脑肿瘤患者,显微镜下手术切除第四脑室肿瘤,其中全切62例,次全切19例,大部切除5例。术后有39例患者行放射治疗。结果86例患者中,3例术后10 d内死亡;83例生存,且症状均有缓解,平均生存期>3年。术后病理诊断髓母细胞瘤32例,室管膜瘤23例,星型胶质细胞瘤15例,血管母细胞瘤10例,脉络丛乳头状瘤2例,表皮样囊肿4例。结论对髓母细胞瘤、星型胶质细胞瘤、血管母细胞瘤应尽可能做到全切,室管膜瘤与脑干粘连紧密者,可考虑次全切除。颅脑恶性肿瘤患者,应结合放疗等综合治疗。     

经小脑延髓裂显微外科切除第四脑室肿瘤（附22例报告）

目的：总结经小脑延髓裂入路显微手术切除第四脑室内肿瘤的手术方法,提高手术治疗效果.方法：回顾性分析2006年3月至2010年9月收治的22例第四脑室肿瘤患者的临床资料.患者均采用枕下后正中切口,打开小脑延髓裂后暴露并显微切除第四脑室肿瘤.1例患者为获得充分显露而切开下髓帆,硬脑膜予以严密缝合或修补.结果：病变全切除19例,近全切除3例.术后病理证实为室管膜瘤10例,星形细胞瘤4例,髓母细胞瘤2例,血管母细胞瘤2例,海绵状血管瘤3例,表皮样囊肿1例.无围手术期死亡,术后患者均未出现与手术入路及术中牵拉有关的并发症.1例患者术后脑积水,行脑室腹腔分流术后好转.结论：经小脑延髓裂入路能较好地暴露第四脑室肿瘤,对小脑损伤小,牵拉轻,是一种安全的手术入路.     

脑室内肿瘤影像学表现及临床特点

目的 探讨不同类型脑室内肿瘤的影像学表现及临床特点.方法 回顾性分析22例经手术病理证实的脑室内肿瘤,总结其MRI、CT影像学表现及临床特点.结果 22例中室管膜瘤7例,脉络丛乳头状瘤、髓母细胞瘤各4例,生殖细胞瘤、中枢神经细胞瘤、室管膜下瘤各1例,室管膜下巨细胞星形细胞瘤和脑膜瘤各2例.男性儿童和青少年患者所占比例较大,发生于侧脑室最多,透明隔病变最少. 脉络丛乳头状瘤仅见于侧脑室内、室管膜瘤第四脑室多见、生殖细胞瘤蝶鞍多见、脑膜瘤侧脑室三角区多见,室管膜下巨细胞星形细胞瘤室间孔区多见. MRI大部分肿瘤T1WI呈等或低信号,T2WI呈稍高信号,增强扫描大部分呈不均匀强化;钙化囊变多见,出血少见;几乎都有脑积水、半数发生脑水肿.结论 脑室内肿瘤有其临床症状特点及影像学征象,MRI及CT对脑室内肿瘤诊断有明显的优势.     

后颅凹占位性病变CT影像学探讨

收集１１７例经手术和病理证实的后颅凹占位病变病例，其中肿瘤１１４例，囊肿３例。髓母细胞瘤、星形细胞瘤及室管膜瘤多发生于青少年。小脑桥脑角好发听神经瘤、脑膜瘤、胆脂瘤，往往有四脑室侧移和后移。小脑蚓部好发髓母细胞瘤，占８２．４％，可引起四脑室前移。四脑室好发室管膜瘤，占８０％，引起四脑室扩大。小脑半球好发血管母细胞瘤、星形细胞瘤及多胶母细胞瘤、转移瘤，往往引起四脑室侧移及前移。后颅凹各种占位性病变都有ＣＴ影像学特征。可依据症状，与四脑室之间的关系，多可准确定位及定性诊断，本文定位诊断符合率为８９．７％。定位诊断准确率提高，可进一步提高定性诊断准确率。     

改良后颅窝正中带蒂骨瓣开颅术

背景与目的:后颅窝肿瘤多采用开骨瓣、硬膜缝合、骨瓣复位方法进行开颅和关颅。本文介绍改良后颅窝带蒂开骨瓣术。方法:小脑蚓部及四脑室肿瘤选择后颅窝正中入路,开骨瓣时采用后正中带蒂骨瓣,术毕骨瓣复位。结果:实施17例后正中开颅肿瘤切除术,小脑蚓部髓母细胞瘤9例、星形细胞瘤8例、第四脑室室管膜瘤3例。术后无皮下积液等并发症。结论:后颅窝正中开颅手术采用带蒂骨瓣、硬膜缝合、骨瓣复位较单纯开瓣术更加符合解剖复位原则,减少颅骨锁等异物植入数量,术后无明显并发症。     

经小脑延髓裂治疗四脑室室管膜瘤

目的：总结第四脑室室管膜瘤的显微外科治疗经验。方法：回顾性分析2000年1月至2010年1月我院收治的50例第四脑室室管膜瘤患者的临床资料。结果：肿瘤全切41例,次全切除5例,大部切除4例,无手术死亡病例,术后发生并发症4例：小脑缄默症2例,脑积水1例,脑神经功能障碍1例。术后随访平均6.5年,其中恢复良好42例,改善5例,恶化3例。结论：应用显微外科技术行经小脑延髓裂入路治疗第四脑室肿瘤,手术成功率高。熟悉第四脑室的显微解剖,注意保护脑干功能是手术成功的关键。     

全中枢神经系统照射治疗脑瘤(附7例报告)

原发于中枢神经系统肿瘤如髓母细胞瘤、幕下室管膜瘤等由于有沿蛛网膜下腔随脑省液循环播散的倾向，在放射治疗中常需要行全中枢神经系统即全脑、全脊髓照射。我院1988年4月至1991年6月共治疗7例，现就治疗技术和疗效报告如下。材料与方法本组男6例，女1例；年龄6～36岁，中位年龄21岁，10岁以下2例。肿瘤类型与部位：髓母细胞瘤5例，其中小脑蚓部2例，小脑半球2例，右侧杨叶1例。室管膜瘤2例，位于第三脑室和右侧脑室1例（室管膜母细胞瘤）和第四脑室1例。肿瘤完整摘除1例，部分切除5例，穿刺活极1例。全部病例放疗前均穿刺抽吸脑脊液细胞…     

髓母细胞瘤的放疗：附17例分析

髓母细胞瘤恶性程度高,术后极易复发,但对射线敏感,我科1962～1984年收治17例髓母细胞瘤术后病例,经全中枢神经系统照射,即:全脑照射DT25～30Gy,后颅凹40～50Gy。全脊髓预防DT30Gy,结果6/17存活5年以上。本瘤常发于小脑蚓部侵犯第四脑室而引起脑脊液循环障碍。为避免在放疗中发生颅压增高,放疗前应先行手术,尽量切除肿瘤。由于瘤细胞易随脑脊液播散,因此应行全中枢神经系统照射。     

显微外科手术联合术后放疗治疗脊髓髓内胶质瘤的临床疗效

目的探讨显微外科手术联合术后放疗治疗脊髓髓内胶质瘤的临床疗效。方法回顾性分析2010年2月至2014年4月在手术治疗的53例脊髓髓内胶质瘤患者的临床资料,分析其疗效。结果 23例室管膜瘤患者中,13例患者行次全切除术,10例患者行全切除术,其中2例患者术后进行放疗治疗。30例星形细胞瘤患者中,12例患者行大部分切除术,18例患者全切除术,30例术后均接受放化疗治疗。53例患者Mccormck分级Ⅰ级10例、Ⅱ级19例、Ⅲ级3例和Ⅳ级3例。改善40例(79.2%),无变化10例(18.9%),加重3例(5.7%)。术后随访6个月至2年,其中1例星形细胞瘤大部分切除患者1年后临床表现症状加重,行磁共振成像检查显示无复发。结论脊髓髓内胶质瘤患者采取显微外科手术联合术后放疗,不仅可以切除肿瘤,还可保证患者的神经功能,提高患者的生活质量,值得临床推广。     

儿童髓母细胞瘤的显微手术治疗——附24例报告

背景与目的：髓母细胞瘤是儿童常见的中枢神经系统恶性肿瘤，手术是其治疗的主要手段。本文通过对24例儿童髓母细胞瘤显微外科治疗分析，总结儿童髓母细胞瘤的显微手术经验。方法：收集1999-2007年我院经手术证实的儿童髓母细胞瘤24例，对其临床资料进行回顾性分析。其中男16例，女8例，平均年龄7．5岁，常见症状和体征为高颅压征和共济失调，全部患者均行显微手术治疗，术后辅助放射治疗。19例随访6个月至6年。结果：全组病例中，肿瘤全切21例，大部分切除2例，部分切除1例，手术死亡率4．2％（1／24），未出现严重并发症．患儿生存质量得到提高。结论：掌握显微手术方法和手术中注意事项，有利于提高肿瘤切除程度及减少手术并发症．手术后辅助放疗有利于改善预后。     

经翼点入路显微手术切除鞍区肿瘤66例临床研究

目的探讨经翼点入路显微手术切除鞍区肿瘤的方法及临床效果。方法对2000年1月至2012年2月收治的66例经翼点入路显微手术切除鞍区肿瘤患者进行回顾性分析总结。结果66例患者,肿瘤全切55例,次全切8例,大部切除3例。术后病理检查,垂体瘤38例,颅咽管瘤16例,脑膜瘤12例;术后32例发生尿崩,18例出现低钠血症,3例高钠血症,无手术死亡病例。54例患者随访6个月至5年,术前视力下降45例,术后视力恢复好转38例,无变化5例,恶化2例。肿瘤残留11例,术后行1刀放疗6例,肿瘤复发3例,1例鞍区并发眶内脑膜瘤。结论在经翼点入路行鞍区肿瘤显微手术中,熟练掌握规范的手术入路和局部显微解剖以及良好的显微外科技术是提高肿瘤全切率和手术疗效的保证。     

The microscopic surgical treatment for tumor of posterior cranial fossa in children

OBJECTIVE To analyze and discuss about the clinical characteristics, pathological types, surgical modalities and techniques, and postoperative complications in children with tumor of posterior cranial fossa.
METHODS Retrospective study was conducted on 102 cases of pediatric tumor of posterior cranial fossa, admitted and treated in our hospital during the period of January 1996 to January 2007. All patients underwent microscopic surgical treatment. Fiftyeight were male and 44 cases were female. The age ranged from 9 months to 14 years old, with an average of 6.1 ± 0.5 of age. Cranial CT or MRI examination was conducted before and after the surgery on all patients. RESULTS The primary manifestations for this group of patients were increased intracranial pressure and/or ataxia. Postoperative pathological diagnoses showed： 46 cases of medulloblastoma, 43 cases of astrocytoma, 11 cases of ependymoma （including 1 case of degenerative ependymoma）, 1 case of dermoid cyst, and 1 case of teratoma. In this group of the patients, radical surgery was used in 68 cases and subtotal surgical removal used in 31 cases, while surgical removal of large section was performed on 3 cases. There were no deaths from surgery reported. Ninety-one cases showed significant symptomatic improvement when compared with preoperative conditions, while 11 cases showed either no improvement or more severely affected afterward. For 6 cases, postoperative ventriculoperitoneal shunt was performed within 7 days to 2 months after the surgery. Sixty-three patients gained follow-up for 3 to 60 months in duration. Thirty-nine patients regained normal life and were able to learn well, while there were 7 patients who could not live normally on their own. During the follow-up period, there were 17 cases of recurrence and 7 cases of death. In 23 cases of medulloblastoma in children with age of 3 years old or above, 2 cases who underwent surgical removal of intracranial ependymoma received small dosage of postoperative X-ray radiotherapy on the the brain and spinal cord. Nine cases of medulloblastoma in children under age of 3 and 17 cases of astrocytoma diagnosed after the surgery received chemotherapy of Carmustine.
CONCLUSION Medulloblastomas and astrocytomas were the most common types of pediatric tumor of posterior cranial fossa, right followed by ependymoma, and dermoid cysts and teratomas were rare. Early correct diagnosis, proper selection of appropriate surgical modality and the surgical margin, proper treatment of postoperative complications, and the selecting right radiotherapy or chemotherapy were the key factors in influencing the prognostic outcome of children with tumor of posterior cranial fossa.     

Treatment and prognosis of patients with intracranial nongerminomatous malignant germ cell tumors: a multiinstitutional retrospective analysis of 41 patients

BACKGROUND: The relative roles of surgical resection, radiotherapy, and chemotherapy in the management of patients with intracranial nongerminomatous malignant germ cell tumors have been controversial. The authors retrospectively investigated the results of different treatment regimens in patients with these tumors. METHODS: The records of 41 patients who were treated between 1981 and 2001 were reviewed. They were grouped into patients with a good prognosis (n=3), an intermediate prognosis (n=24), and a poor prognosis (n=14) based on the histology of their tumors. Fifteen patients (37%) underwent surgical resection and received radiotherapy, and 26 patients (63%) also received chemotherapy. The median follow-up of 18 patients who remained alive was 61 months (range, 14-194 months). RESULTS: The 5-year actuarial overall survival rates for patients in the good prognosis, intermediate prognosis, and poor prognosis groups were 100%, 68%, and 8%, respectively. In the analysis, histology alone had a statistically significant impact on overall survival (P<0.0001). All 3 patients in the good prognosis group were treated successfully with surgical resection and radiotherapy. In the intermediate prognosis group, the 5-year actuarial overall survival rate was 44% for patients who underwent surgical resection and received radiotherapy (n=9) and 84% for patients who also received chemotherapy (n=15; P=0.01). Patients in the poor prognosis group who underwent surgical resection and received radiotherapy (n=3) or who underwent incomplete resection and received both radiotherapy and chemotherapy (n=8) all died of disease, whereas 2 of 3 patients who underwent macroscopic total resection and received both radiotherapy and chemotherapy survived free of disease. CONCLUSIONS: The treatment of patients with intracranial nongerminomatous malignant germ cell tumors should be based on tumor histology. For patients who had a good prognosis (mature teratoma with germinoma), surgical resection and radiotherapy were sufficient; however, for patients in the intermediate prognosis group, multimodal treatment, including surgical resection, radiotherapy, and chemotherapy, was effective. Conversely, for patients in the poor prognosis group, more intensive multimodal treatment, including macroscopic total resection, may improve the survival rate.     

The Microscopic Surgical Treatment for Tumor of Posterior Cranial Fossa in Children

OBIECTIVE To analyze and discuss about the clinical characteristics,pathological types,surgical modalities and techniques,and postoperative complications in children with tumor of posterior cranial fossa.METHODS Retrospective study was conducted on 102 cases of pediatric tumor of posterior cranial fossa,admitted and treated in our hospital during the period of January 1996 to January 2007.All patients underwent microscopic surgical treatment.Fiftyeight were male and 44 cases were female.The age ranged from 9months to 14 years old,with an average of 6.1±0.5 of age.Cranial CT or MRI examination was conducted before and after the surgery on all patients.RESULTS The primary manifestations for this group of patients were increased intracranial pressure and/or ataxia.Postoperative pathological diagnoses showed:46 cases of medulloblastoma,43cases of astrocytoma,11 cases of ependymoma(including 1 case of degenerative ependymoma),1 case of dermoid cvst,and 1 case of teratoma.In this group of the patients,radical surgery was used in 68 cases and subtotal surgical removal used in 31 cases,while surgical removal of large Section was performed on 3 cases.There were no deaths from surgery reported.Ninety-one cases showed significant symptomatic improvement when compared with preoperative conditions,while 11 cases showed either no improvement or more severely affected afterward.For 6 cases,postoperative ventriculoperitoneal shunt was performed within 7 days to 2 months after the surgery.Sixty-three patients gained follow-up for 3 to 60 months in duration.Thirty-nine patients regained normal life and were able to learn well,while there were 7 patients who could not live normally on their own.During the follow-up period,there were 17 cases of recurrence and 7 cases of death.In 23 cases of medulloblastoma in children with age of 3 Years old or above,2 cases who underwent surgical removal of intracranial ependymoma received small dosage of postoperative X-ray radiotherapy on the the brain and spinal cord.Nine cases of medulloblastoma in children under age of 3 and 17 cases of astrocytoma diagnosed after the surgery received chemotherapy of C:armustine.CONCLUSION Medulloblastomas and astrocytomas were the most common types of pediatric tumor of posterior cranial fossa,right followed by ependymoma,and dermoid cysts and teratomas were rare.Early correct diagnosis,proper selection of appropriate surgical modality and the surgical margin,proper treatment of postoperative complications,and the selecting right radiotherapy or chemotherapy were the key factors in influencing the prognostic outcome of children with turrtor of posterior cranial fossa.     

单纯Barrett's食管腺癌108例临床分析

目的分析单纯Barrett’s食管腺癌根治性手术切除后加用放、化疗的临床疗效,探讨影响Barrett’s食管腺癌预后的因素。方法回顾性分析108例Barrett’s食管腺癌患者的临床资料及随访结果。行根治性手术切除者92例,其中术后单纯放疗者76例,术后放疗后加用化疗者16例;姑息性手术治疗者16例,其中有4例患者于术中死亡,另12例患者在术后均采用放疗 化疗。放疗采用60钴或6MV-X线,体外常规分割照射,靶区放射治疗总剂量为55～70 Gy;化疗采用FAM或CMF方案4～6个疗程。结果采用Kaplan-Meier法计算其生存率,本组患者1、3、5年生存率分别为81.5%、51.9%和22.2%,其中根治性手术切除后单纯放疗者5年生存率为15.8%,术后放疗加化疗者为75.0%;有肿瘤外侵者5年生存率为9.1%,无肿瘤外侵者为33.3%;有淋巴结转移者5年生存率为14.3%,无淋巴结转移者为33.3%;姑息性手术治疗术后加放、化疗后5年生存率为0。Log rank检验结果显示,差异均有统计学意义(P<0.05)。结论单纯Barrett’s食管腺癌根治性手术切除后,及时加用放、化疗治疗,有助于疗效的提高。影响预后的主要因素为临床分期、肿瘤外侵、淋巴结转移和根治性手术切除后放、化疗措施的应用。     

Treatment of medulloblastoma with postoperative chemotherapy alone: an SFOP prospective trial in young children

BACKGROUND: Morbidity and mortality are high in young children with medulloblastoma who receive craniospinal radiotherapy. We aimed to assess whether adjuvant treatment with protracted chemotherapy alone could replace radiotherapy. METHODS: We enrolled 79 children aged younger than 5 years who had had surgical resection of medulloblastoma onto a multicentre trial. Patients were treated with combination chemotherapy, which did not include methotrexate, for more than 16 months irrespective of the extent of disease. Early postoperative imaging defined three groups: R0M0 (no residual disease, no metastasis), R1M0 (radiological residual disease alone), and RXM+ (presence of metastases). Patients who did not relapse did not receive radiotherapy. Patients who relapsed or had disease progression received salvage treatment, which consisted of high-dose chemotherapy and stem-cell transplantation followed by local or craniospinal radiotherapy. For children classified as R0M0, the primary endpoint was 5-year overall survival and the secondary endpoint was 5-year progression-free survival. For children classified as R1M0 or RXM+, the primary endpoint was best radiological response and the secondary endpoints were 5-year overall survival and 5-year progression-free survival. Analyses were done by intention to treat. FINDINGS: Two of 15 patients classified as RXM+ and four of 17 patients classified as R1M0 had a complete radiological response. 5-year progression-free survival was 29% (95% CI 18-44) in the R0M0 group, 6% (1-27) in the R1M0 group, and 13% (4-38) in the RXM+ group. 5-year overall survival was 73% (59-84) in the R0M0 group, 41% (22-64) in the R1M0 group, and 13% (4-38) in the RXM+ group. In the R0M0 group, 5-year progression-free survival was 41% (26-58) for the 34 patients who underwent gross total resection compared with 0% for the 13 patients who had subtotal resection (relative risk 2.7 [1.3-5.6], p=0.0065). INTERPRETATION: Conventional chemotherapy alone can be used to cure children with non-metastatic medulloblastoma who have gross total resection confirmed by early radiological assessment, but is not sufficient for treatment of those with metastatic or incompletely resected medulloblastoma. Salvage treatment followed by posterior-fossa radiotherapy can effectively treat local relapses or progression.     

Risk-adapted craniospinal radiotherapy followed by high-dose chemotherapy and stem-cell rescue in children with newly diagnosed medulloblastoma (St Jude Medulloblastoma-96): long-term results from a prospective, multicentre trial

BACKGROUND: Current treatment for medulloblastoma, which includes postoperative radiotherapy and 1 year of chemotherapy, does not cure many children with high-risk disease. We aimed to investigate the effectiveness of risk-adapted radiotherapy followed by a shortened period of dose-intense chemotherapy in children with medulloblastoma. METHODS: After resection, patients were classified as having average-risk medulloblastoma (< or = 1.5 cm2 residual tumour and no metastatic disease) or high-risk medulloblastoma (> 1.5 cm2 residual disease or metastatic disease localised to neuraxis) medulloblastoma. All patients received risk-adapted craniospinal radiotherapy (23.4 Gy for average-risk disease and 36.0-39.6 Gy for high-risk disease) followed by four cycles of cyclophosphamide-based, dose-intensive chemotherapy. Patients were assessed regularly for disease status and treatment side-effects. The primary endpoint was 5-year event-free survival; we also measured overall survival. This study is registered with ClinicalTrials.gov, number NCT00003211. FINDINGS: Of 134 children with medulloblastoma who underwent treatment (86 average-risk, 48 high-risk), 119 (89%) completed the planned protocol. No treatment-related deaths occurred. 5-year overall survival was 85% (95% CI 75-94) in patients in the average-risk group and 70% (54-84) in those in the high-risk group (p=0.04); 5-year event-free survival was 83% (73-93) and 70% (55-85), respectively (p=0.046). For the 116 patients whose histology was reviewed centrally, histological subtype correlated with 5-year event-free survival (p=0.04): 84% (74-95) for classic histology, 77% (49-100) for desmoplastic tumours, and 57% (33-80) for large-cell anaplastic tumours. INTERPRETATION: Risk-adapted radiotherapy followed by a shortened schedule of dose-intensive chemotherapy can be used to improve the outcome of patients with high-risk medulloblastoma.