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。     

儿童髓母细胞瘤诊治进展

髓母细胞瘤的手术方式和放化疗策略的不断优化使多数患儿的治疗和预后得到明显提高,但近年研究证明预后不仅仅和手术以及放化疗的策略有关,而且和肿瘤多样化的内在生物学特性有密切联系,一些生物学标志物对肿瘤的诊断、治疗方式选择和预后评价有重要意义。髓母细胞瘤的复发和转移以及婴幼儿的治疗仍是难点,改善低危患儿长期预后和进一步提高高危患儿生存率将是未来研究的课题。     

儿童髓母细胞瘤诊治进展

髓母细胞瘤的手术方式和放化疗策略的不断优化使多数患儿的治疗和预后得到明显提高，但近年研究证明预后不仅仅和手术以及放化疗的策略有关，而且和肿瘤多样化的内在生物学特性有密切联系，一些生物学标志物对肿瘤的诊断、治疗方式选择和预后评价有重要意义。髓母细胞瘤的复发和转移以及婴幼儿治疗仍是难点，改善低危患儿长期预后和进一步提高高危患儿生存率将是未来研究的课题。     

儿童髓母细胞瘤的综合治疗

儿童中枢神经系统原发肿瘤占所有儿童期肿瘤的 2 0 % ,发生率仅次于白血病居于第二位。髓母细胞瘤(ｍｅｄｕｌｌｏｂｌａｓｔｏｍａＭＢ)占儿童原发脑肿瘤的 2 0 % ,其发生率仅次于星形细胞瘤[1] 。发病高峰年龄在 7岁左右。髓母细胞瘤具有高度的软脑膜转移倾向 ,尤其在低龄儿童中常见。髓母细胞瘤 (ＭＢ)起源于小脑蚓部向四脑室生长 ,是最常见的儿童原发恶性脑肿瘤 ,是中枢神经系统恶性程度最高的神经上皮性肿瘤之一 ,属于原始神经外胚层肿瘤 (ｐｒｉｍｉｔｖｅｎｅｕｒｃｅｐｉｔｈｅｌｉａｌｔｕｍｏｒｓＰＮＥＴＳ)的一种 ,在ＷＨＯ的神…     

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。     

儿童髓母细胞瘤的治疗

报道２～１５岁儿童髓母细胞瘤５４例，６例因肿瘤转移他处死亡，２例因肿瘤出血死亡，４６例采用手术治疗，手术死亡２例，死亡率为４．４％。全切除肿瘤３９例，部分切除３例，亚全切除４例，随访４０例，１例因肿瘤属Ｔ－４级死亡，临床治愈２７例，症状改善１２例，５年生存率为５０％，推荐放射治疗为首选，就诊断及治疗进行了讨论。     

儿童髓母细胞瘤基础研究进展

髓母细胞瘤(medulloblastoma,MB)是儿童中枢神经系统最常见的恶性肿瘤。根据2007年WHO中枢神经系统肿瘤分类分为四种亚型:促纤维增生/结节型髓母细胞瘤、髓母细胞瘤伴广泛结节、间变性髓母细胞瘤、大细胞型髓母细胞瘤。它     

显微外科治疗儿童髓母细胞瘤

目的 用显微技术切除儿童髓母细胞瘤,砬少手术并发症,提高术后生存质量。方法 对３４例儿童髓平细胞瘤的临床资料进行分析。结果 ３４例均采用显微手术治疗,全切２７例,次全切除４例,部分切除３例,手术死亡２例,死亡率为５．９％,获随该列,临床治愈２４例,着状改善６例,５年生存率为５０％。结论 微创的显微外科技术是保证减少术后并发症,获得良好预后的关键。对于大多数患儿,建议术后辅以放疗。     

髓母细胞瘤的现状和未来

髓母细胞瘤是一种多发于小儿的肿瘤,其主要特点是易沿脑脊液通路播散。随着颅脊柱放疗前后化疗的应用,患者五年存活率已达到60％。本文简要综述了该病的病理学和生物学特征,以及治疗和实验研究进展。     

三维适形放疗同期鞘内注射化疗后全身化疗治疗高危髓母细胞瘤

目的 总结全程三维适形放疗(3DCRT)同期鞘内注射化疗后全身化疗治疗高危髓母细胞瘤(MB)的疗效. 方法 南方医科大学珠江医院肿瘤中心自2005年1月至2008年1月行3D CRT联合鞘内注射及全身化疗治疗已沿脑脊液播散的高危MB患者20例,其中脑脊液中找到肿瘤细胞15例,MRI发现脊髓结节状种植5例.采用3DCRT进行全脑全脊髓放疗(CSI)及局部追量,放疗同期应用阿糖胞苷、氨甲喋呤等鞘内注射,放疗后应用盐酸尼莫司汀、替莫唑胺等全身化疗,回顾性分析患者的临床资料并总结疗效. 结果 本组患者中位随访时间48.5个月.治疗结束3个月后患者总体完全缓解(CR) 18例(90.0％),部分缓解(PR)1例(5.0％),稳定(SD)1例(5.0％);4例治疗失败死亡.1例局部复发.3例脊髓种植进展.患者中位生存时间53个月,3年总生存率(OS)和3年无病生存率(DFS)分别为80.0％、75.5％.不良反应主要为血液、消化系统的毒性反应,未出现Ⅳ度骨髓抑制. 结论 全程3DCRT同期鞘内注射化疗后全身化疗治疗高危MB安全、有效,预后好.     

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.     

Prognostic role of chemotherapy,radiotherapy dose,and extent of surgical resection in adult medulloblastoma

PurposeAdult medulloblastoma is rare, and management is extrapolated from pediatric cases. This investigation evaluated the prognostic role of chemotherapy (and sequencing thereof), the degree of resection, and craniospinal irradiation (CSI) dose.MethodsThe National Cancer Database was queried for adult (age ≥18) medulloblastoma. Resection was coded as gross (GTR) or subtotal resection (STR) or biopsy only; concurrent chemoradiotherapy (CRT) was defined as receipt within 14 days of each other. Statistics included Kaplan-Meier overall survival (OS) analysis and Cox proportional hazards modeling.ResultsOf 1144 patients, 613 had coded surgical information; 242 (39%) did not undergo surgery, 277 (45%) underwent STR, and 94 (15%) had GTR. A total of 428 (37.4%) did not receive chemotherapy, 348 (30.4%) received sequential CRT, and 368 (32.2%) underwent concurrent CRT. Of the 711 patients with CSI dose information, 202 (28.4%) received 23–30 Gy CSI and 509 (71.6%) patients received 30–36 Gy. Median follow-up was 56.5 months. Extent of resection did not correlate with 10-year OS (74.2% biopsy only, 72.7% STR, 82.2% GTR, p > 0.05 all comparisons) or on Cox multivariate analysis. Chemotherapy was associated with higher OS (65.6% vs. 51.2%, p = 0.035) and a trend towards significance on multivariate assessment (p = 0.082). Sequencing of chemotherapy and CSI dose were not associated with OS (p > 0.05 for both).ConclusionsAlthough causation cannot be implied, neither the extent of resection nor CSI dose associated with OS in adult medulloblastoma. Chemotherapy could have utility in higher-risk patients; concurrent administration may not be beneficial, especially given therapy-induced neuro-cognitive sequelae.     

Modern treatment strategies in medulloblastoma

Chemotherapy has an important role in the modern treatment of children with medulloblastoma (MB). In patients at high risk, intense chemotherapy should improve the survival rate. In low-risk patients chemotherapy should allow the dose of craniospinal irradiation to be reduced, which in turn should improve the quality of life. In infants under 3 years of age radiotherapy should be delayed, or even replaced by postoperative chemotherapy. Chemotherapy is also necessary to prevent or treat systemic dissemination. The optimal timing of chemotherapy is a focus of contemporary research.     

化、放疗联合治疗儿童颅内生殖细胞瘤34例临床随诊观察

目的：观察颅内生殖细胞瘤经过化疗并辅以中低剂量放疗后的疗效及对儿童生长发育的影响。方法：自1993年12月至2001年12月共对63例颅内生殖细胞瘤的儿童进行静脉化疗并辅以中低剂量放疗，随访到34例，其中肿瘤位于松果体区20你，松果体区并鞍区6例，鞍区4例，松果体区并脑室内3例，底节区1例。使用药物为长春新碱、甲氨蝶呤、平阳霉素及顺铂。用药后每周化验2次血常规，1次血生化，以观察周围血象变化及心、肝、肾功能情况，并进行针对性治疗。4周后如血常规及血生化的各项指标均正常，再行第二疗程化疗。2个疗程结束后1个月，在肿瘤病灶局部补充中低剂量（25-35Gy）的放疗。每个疗程间进行CT或MRI检查，观察肿瘤消退的情况。：34例患者随访最短1年，最长7年。1例为手术加全剂量放疗后2年肿瘤复发，此次化疗进行2个疗程，但化疗后未再补充放疗，4年后肿瘤复发，家属放弃治疗，患者死亡。1例化、放疗后5年肿瘤复发，再行2疗程化疗，疗效较首次治疗差，其余32例（94％）化、放疗后1-7年，复查MRI显示肿瘤无复发，患者生存质量良好，儿童生长发育及智力均未受影响。结论：静脉化疗辅以中低剂量放疗应为治疗儿童颅内生殖细胞瘤的最佳方案。     

Outcome of medulloblastoma in children treated with reduced-dose radiation therapy plus adjuvant chemotherapy

Medulloblastoma is the most common malignant brain tumor in children. Post-surgical craniospinal irradiation (CSI; 30-36 Gy) plus local boost radiation therapy (RT; 54-56 Gy) is a standard treatment for children with medulloblastoma who are over 3 years old, resulting in a 5-year overall survival (OS) rate of 46% to 65% in average-risk patients and 50% in high-risk patients. The addition of chemotherapy has the benefit of reducing complications from radiation and improving the OS rate. Using this approach, the estimated 5-year OS rates for patients with average- and high-risk medulloblastomas treated with different protocols are 65% to 85% and 16% to 70%, respectively. In this study, we determined the outcome of patients with average- and high-risk medulloblastomas treated with reduced dosage CSI and chemotherapy with an oral etoposide-based regimen. The study included 49 patients, with a mean age of 7.7 ± 3.4 years. Twenty-six patients (53%) were classified as average-risk and 23 patients (47%) as high-risk. In the average-risk group, the 5-year progression free survival (PFS) rate was 62.9% ± 10% and the 5-year OS rate was 70.4% ± 9.5%. In the high-risk group the 5-year PFS rate was 48.9% ± 13% and the 5-year OS rate was 49.7% ± 13%. In the average-risk group, patients who received CSI of either 24 Gy (n = 20) or 36 Gy (n = 9) showed no difference in their 5-year PFS and OS rates. We found that patients who were ?10 years old and patients who were female had a significantly better 5-year PFS rate.     

Neurological complications of radiotherapy and chemotherapy

Neurological complications of radiotherapy and chemotherapy can affect the central or peripheral nervous system. Most are dose-dependent and constitute a limiting factor in the administration of treatments. Radiation-induced neurological complications are classified as acute, early-delayed or delayed. The most important are radionecrosis and cognitive dysfunction/leukoencephalopathy. Neurotoxicity of chemotherapy is frequent and depends upon dose, type of drugs (especially cisplatin and methotrexate) and their combination with radiotherapy. Received: 6 March 1998 Accepted: 28 March 1998     

Multidisciplinary treatment of medulloblastomas in childhood

The analysis of 65 medulloblastomas in children treated at the National Institute of Pediatrics, Mexico City, between 1971 and 1986 is reported. The patients were staged retrospectively. Ninety percent presented without metastasis in the subarachnoid space or the spinal fluid. Following surgery, all patients underwent radiotherapy to the brain, posterior fossa, and spinal cord. For the last 35 patients, chemotherapy was added to the treatment regimen. The actuarial survival was 55% at 6 years in the group with chemotherapy versus 27% to the group without chemotherapy, with a statistically significant difference (P<0.01).     

基底神经节区生殖细胞瘤的早期诊断及综合治疗

目的 探讨颅内基底神经节区生殖细胞瘤的早期诊断及综合治疗.方法 对25例颅内基底神经节区生殖细胞瘤患者的年龄、性别、发病早期的临床症状、体征、血液中肿瘤标记物的榆查及影像学检查的结果等特点进行分析判断,得出早期诊断的依据并行早期综合治疗.结果 3例开颅手术,1例活榆,病理证实为生殖细胞瘤,21例通过临床特点分析,在生殖细胞瘤早期即明确诊断,末行手术,直接进行化、放疗.患者随访最长7年,最短10个月,平均45个月.1例外院手术大部分切除肿瘤,病理诊断生殖细胞瘤,来我院行化、放疗,6年后肿瘤复发,再次化疗,肿瘤消失,已随访1年未见复发,其余24例随访均未见肿瘤复发.患者经过治疗后,肿瘤虽已消失,但症状、体征并不能完全缓解.结论 通过细致的临床观察,综合分析判断临床特点,基底神经节区生贿细胞瘤是可以做到早期诊断,并通过化、放疗等非手术治疗达到满意的治疗效果.