57例髓母细胞瘤的临床回顾性分析

目的 研究髓母细胞瘤的临床特点及显微外科手术治疗的I临床疗效. 方法 回顾性分析经显微手术治疗的57例髓母细胞瘤患者的临床和随访资料.对髓母细胞瘤的显微外科手术技巧进行探讨. 结果 肿瘤全切除42例,近全切除13例.部分切除2例.全部病例均打通中脑导水管.术前有脑积水者43例,术后手术疗效明显,脑积水减少至16例.且较术前均有不同程度改善,其中10例术后需行分流术.术后两年内肿瘤复发19例,中枢神经系统种植转移8例.最早术后20 d肿瘤复发.术后2年生存率68.4%(截至2006年7月病例),术后5年生存率49.1%(截至2003年7月病例). 结论 应用显微外科手术全切除肿瘤和术后全中枢轴放疗可使髓母细胞瘤患者取得较好的临床疗效.     

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。     

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。     

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

目的　分析总结成人髓母细胞瘤的临床特点及治疗效果。方法　经手术及病理证实的成人髓母细胞瘤５３ 例, 其中男性３６ 例, 女性１７ 例, 平均年龄２５８ 岁。肿瘤均位于小脑, 蚓部２９ 例,小脑半球２４ 例, 伴鞍上转移１ 例。结果　全切２０ 例, 近全切除２０ 例, 大部切除１２ 例, 部分切除１例, 手术死亡率１９ ％ 。随访到４０ 例, 均已行放疗, 复发１７ 例, 中枢神经系统种植转移４ 例, ５ 年存活率４０ ％ 。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, 一旦出现种植转移, 则预后差。     

髓母细胞瘤的综合治疗（附55例分析）

目的探讨髓母细胞瘤的显微切除技术及手术加放疗及化疗的综合疗效。方法回顾性分析55例髓母细胞瘤病人的临床资料。均采用显微手术切除,手术后采用术野区域分次放疗,平均剂量30Gy,不作常规脊髓、全脑放射。化疗方案：长春新碱（0.05mg/kg,每周1次,3次/疗程）＋顺铂（3.5mg/kg,1次/疗程）＋环磷酰胺（65mg/kg,2次/疗程）＋氨甲蝶呤（400mg/kg,1次/疗程）,共4个疗程。结果肿瘤全切除45例,次全切除6例,大部切除4例;术后无明显神经功能损36例,出现轻度后组脑神经核性损伤11例,中～重度损伤8例。病人3、5、10年生存率分别为72%、58%、22%。平均肿瘤复发时间42个月。结论对髓母细胞瘤全切除肿瘤、缩短术后放疗与化疗的间隔时间、个性化放化疗方案是获得良好临床预后的关键。     

髓母细胞瘤复发、播散性种植及预后的相关因素分析

目的探讨影响髓母细胞瘤术后局部复发、播散性种植和预后的临床因素。方法回顾性分析我科收治并经病理确认的43例髓母细胞瘤患者,采用Log-Rank法分析肿瘤的T分期、切除程度、放疗方式对患者术后肿瘤复发、播散性种植及生存时间的影响。结果获得随访的41例患者中,3年、5年总体生存率分别为68.3%、50.1%,肿瘤的T分期、切除程度、放疗方式对患者术后复发时间、术后总生存时间的影响均存在统计学差异;肿瘤的T分期、放疗方式对患者播散性种植时间的影响存在统计学差异,而切除程度对其影响无明显统计学差异。结论处于T1、T2期的肿瘤、接受全脑-脊髓放疗的患者手术距离发生复发、播散性种植的时间和术后总生存时间较长,手术全切除能延缓术后复发和延长术后生存时间,但对发生播散性种植的时间无影响。     

儿童髓母细胞瘤显微手术治疗及术后并发症探讨

目的探讨儿童髓母细胞瘤显微手术及术后并发症问题。方法回顾性分析经小脑蚓部切开联合四脑室正中孔入路显微手术切除儿童髓母细胞瘤及术后并发症53例。全部经病理证实。结果全部病例术前均行MRI检查,46例同时行CT检查。手术全切除47例(88.7%)、次全切除6例(11.3%)。术后51例高颅压症状消失,临床体征改善,2例死亡。术后发生咸默症5例,其中合并下颏关节脱位1例。术后昏迷2例,呼吸机辅助人工呼吸2周1例,遗留一侧肢体轻瘫2例。中枢性面瘫2例。肿瘤位于小脑蚓部12例,四脑室内18例。小脑蚓部和四脑室内者23例。肿瘤血供来源于小脑后下动脉37例。全部病例均打通中脑导水管。术后37例患者随访6个月至5年3个月。35例接受放疗。其中30例未见复发和转移。4例原位复发,2例脊髓播种性转移。结论儿童髓母细胞瘤由于其临床特点诊断多无困难,但个性化深入了解肿瘤病理解剖及其与周围组织比邻有利于最大限度切除肿瘤。采用显微手术全切除肿瘤组织、解除导水管梗阻、妥善保护小脑后下动脉及其分支、避免伤及脑干及四脑室底是手术操作的关键。病程进展迅速,肿瘤巨大者术后生存质量及生存期更差,早期就诊尤为重要。     

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。     

48例原发性胶质母细胞瘤的治疗经验

目的探讨胶质母细胞瘤手术加放疗、化疗综合治疗的疗效。方法 2007～2009年收治胶质母细胞瘤患者48例,均采用显微手术切除肿瘤;术后行全脑分割剂量放疗,部分患者采用立体定向放疗;同时联合化疗,方案是静脉滴注替尼泊甙60mg/(m2·d)+司莫司汀100mg/d,或口服替莫唑胺75mg(/m2·d)。结果肿瘤全切除43例,次全切除5例;术后无新增神经功能损伤33例,出现新的失语症状11例,新的肢体偏瘫症状10例。患者1年生存率为56.25%,肿瘤复发时间平均6.8个月。结论全切除肿瘤+术后早期行放疗+个性化化疗可延长胶质母细胞瘤患者生存期。     

Metastatic medulloblastoma in an adult; treatment with temozolomide

Medulloblastoma is a malignant brain tumour most frequently seen in children. Treatment of this tumour type usually consists of surgery followed by radiotherapy. Relapses of medulloblastoma are sensitive to chemotherapy and treatment with chemotherapeutics in children has increased the survival rates. A medulloblastoma at adult age is extremely rare, and there is no overall accepted treatment, especially not in the case of a relapse. Recently improvement of survival was reported in patients with glioblastoma treated with a combination of radiotherapy and concomitant temozolomide. This observation encouraged us to decide to treat an adult patient with a recurrent medulloblastoma with temozolomide. This female patient showed a recurrence of a medulloblastoma 7 years after the initial presentation with metastatic spread along the neuraxis and progressive neurological deterioration. Treatment with temozolomide resulted in relief of clinical symptoms and stabilization of tumour growth for 8 months.     

成人髓母细胞瘤患者的预后影响因素分析

目的探讨成人髓母细胞瘤(MB)患者性别、肿瘤部位、免疫组化指标、是否全切、辅助放化疗等临床、病理因素对预后的影响。方法回顾性分析25例成人髓母细胞瘤患者的临床资料,并对患者的预后进行电话随访。使用Kaplan-Meier曲线和Log-rank检验进行生存分析,寻找影响预后的因素。结果本组患者中,男性14例(56.0%),女性11例(44.0%),平均年龄为(25.1±5.9)岁。其中,12例患者(48.0%)的肿瘤位于小脑内,13例患者(52.0%)肿瘤突入四脑室;20例患者(80.0%)实现了肿瘤全切;23例患者(92.0%)术后进行了放疗;13例患者(52.0%)术后行化疗。本组患者的5年无进展生存和整体生存率分别为(65.9±10.6)%和(67.5±15.6)%。辅助放疗(P<0.001)和突触素阳性表达(P=0.017)与较好的5年无进展生存相关。在术后行辅助放疗的基础上,再行辅助化疗并无确切疗效(P=0.202)。结论成人髓母细胞瘤患者术后行辅助放疗是必需的,是否行辅助化疗尚存争议。辅助放疗和突触素阳性表达与较好的预后相关。免疫组化指标对评估成人髓母细胞瘤患者的预后有价值。     

Outcome of children with posterior fossa medulloblastoma: a single institution experience over the decade 1994–2003

Aim While the impact of radiotherapy in the management of medulloblastoma was recognised, the introduction of chemotherapy was investigated in clinical trials and shown to confer an additional advantage. We reviewed the outcome of a series of consecutive patients to assess the impact in a population-based clinical establishment. Materials and methods A series of 38 children treated for medulloblastoma at Birmingham Children’s Hospital between 1994 and 2003 was analysed. The effect of surgery, radiotherapy, chemotherapy and metastasis on survival was analysed. Results The overall 5-year survival rate was 61.4% for the 36 patients who had resective surgery, while 2 patients had biopsy only and died within a few months. There was no operative mortality. The incidence of hydrocephalus needing permanent shunting was higher in the first 3 years of life (p = 0.007, chi-square). The 5-year survival rate of patients with total and sub-total excision of medulloblastoma was 61.1% and 61.8%, respectively. The 5-year survival rate of patients older than 3 years was 73.4% and for patients under 3 years was 36.3% (p = 0.007, log rank). Metastases at presentation did not influence survival. All deaths occurred in the first 32 months. Conclusion The contribution of chemotherapy in the improvement of the overall survival appears more evident in children younger than 3 years or presenting with metastases. The absence of significant difference in survival between patients with total or sub-total excision of medulloblastoma supports the view that total excision of medulloblastoma can be avoided when the risk for potential intra-operative damage and consequent neurological deficits is high.     

Medulloblastoma with lipidized cells versus lipomatous medulloblastoma

BACKGROUND: Lipomatous medulloblastoma is a recently identified clinicopathological entity, characterized by areas of lipomatous differentiation, manifestation in adults, and apparently by a favorable prognosis. MATERIAL AND METHODS: In our series of medulloblastomas of adults and children we have found lipidized cells within the tumor in 6 out of 78 cases of adults and in 8 out of 44 cases of children. In 3 adult cases and 3 children cases, lipidized cells were particularly numerous and clustered. RESULTS: Neuronal differentiation was found in 4/6 cases; no case showed GFAP-positive tumor cells. Lipidized cells were constantly immunopositive for vimentin and some of them also for KP-1 and CR3/43. The proliferation potential was evaluated by the immunohistochemical demonstration of MIB-1; MIB-1-labeling index (LI) ranged from 20.8% to 40.5%. No case survived longer than 7 years after diagnosis and postoperative radiotherapy. CONCLUSION: The present 6 cases of heavily lipidized medulloblastoma are not uniform as for age of occurrence, proliferation potential and survival. They do not share the clinical and pathologic features of "lipomatous medulloblastoma". Therefore, the finding of large numbers of lipidized cells in a medulloblastoma does not authorize to diagnose the tumor as "lipomatous medulloblastoma", for which a favorable clinical prognosis is foreseen.     

手术联合放化疗治疗儿童髓母细胞瘤的疗效分析

目的 探讨手术联合放化疗治疗儿童髓母细胞瘤（MB）的疗效。方法 回顾性分析2017年1月至2020年7月收治的32例儿童MB的临床资料。均采用枕下后正中入路手术切除肿瘤,术后行放化疗。结果 肿瘤完全或近完全切除26例,次全切除6例。术后发生小脑缄默症7例,硬膜外血肿1例,后组颅神经受损5例,皮下积液3例。32例随访止2020年12月,随访时间9～38个月,平均23.9个月,次全切除6例出现进展,其中脊髓转移3例,死亡5例;存活27例中,26例正常生活,1例因肿瘤进展需要照顾。结论 对于儿童MB,手术联合术后足疗程、足量放化疗,延缓肿瘤复发,提高生存率。     

Adult medulloblastoma: clinical profile and treatment results of 18 patients.

The objective of this article is to examine the clinicoradiological features and surgical outcomes of adult patients (>16 years) with medulloblastoma. An attempt was made to identify the predictors of poor outcome and assess patterns of relapse and to compare these with pediatric medulloblastoma. Retrospective case record analyses were performed on 18 adults (>16 years) and 79 children (<16 years) operated upon after January 1990, who had at least 5 years of follow-up. The following variables were assessed by bivariate analyses: age, location of the lesion, brainstem invasion, extent of excision and histological subtype. Statistical analysis was performed using chi-square test, Fischer's exact test and Student's t-test. Results revealed there was no gender preference. The tumor was located in the vermis in 12 patients (66.6%) and in the cerebellar hemisphere in six (16.6%). Calcification was observed in two cases (11.1%) and hydrocephalus was seen in six (33.3%). MRI evidence of brainstem infiltration was seen in three patients (16.6%). Total excision was achieved in 13 cases (72.2%), near total excision in four (22.2%) and subtotal excision in one. Major complications included fresh cranial nerve deficits in four patients (22.2%), hemiparesis and gait unsteadiness in three (16.6%), mutism in one and meningitis in two. All patients underwent adjuvant radiotherapy in the form of craniospinal irradiation with a posterior fossa boost. On follow-up, 11 patients (61.1%) were observed to have recurrence and all were located in the posterior fossa. All 11 subsequently underwent chemotherapy. Two patients had re-operation for residual/recurrent lesions causing raised intracranial symptoms. Five-year follow-up data showed that 10 patients (55.5%) were alive, five (27.7%) had died while three (16.6%) were lost to follow-up. In spite of recent advances in management, patients with medulloblastoma still have a poor prognosis. However, adults fared better than children. Vermian location had a better outcome in adults, but not in children. Desmoplastic variant histology was not observed to be a significant prognostic factor in the adult group while brain stem invasion carried a poor prognosis.