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后颅窝髓母细胞瘤患儿的治疗结果:单机构1994 - 2003年十年间的经验

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

作者信息

Kombogiorgas D, Sgouros S, Walsh A R, Hockley A D, Stevens M, Grundy R, Peet A, English M, Spooner D

机构信息

Department of Neurosurgery, Birmingham Children's Hospital, Birmingham, UK.

出版信息

Childs Nerv Syst. 2007 Apr;23(4):399-405. doi: 10.1007/s00381-006-0258-5. Epub 2006 Nov 22.

Abstract

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.

摘要

目的

虽然放射治疗在髓母细胞瘤治疗中的作用已得到认可,但化疗的引入在临床试验中得到了研究,并显示具有额外的优势。我们回顾了一系列连续患者的治疗结果,以评估在一家基于人群的临床机构中的治疗效果。

材料与方法

分析了1994年至2003年间在伯明翰儿童医院接受髓母细胞瘤治疗的38例儿童。分析了手术、放疗、化疗和转移对生存的影响。

结果

36例行切除手术的患者5年总生存率为61.4%,2例仅行活检的患者在数月内死亡。无手术死亡。1岁前需要永久性分流的脑积水发生率更高(p = 0.007,卡方检验)。髓母细胞瘤全切和次全切患者的5年生存率分别为61.1%和61.8%。3岁以上患者的5年生存率为73.4%,3岁以下患者为36.3%(p = 0.007,对数秩检验)。就诊时出现转移不影响生存。所有死亡均发生在最初32个月内。

结论

化疗对总体生存改善的贡献在3岁以下或出现转移的儿童中似乎更为明显。髓母细胞瘤全切和次全切患者的生存率无显著差异,这支持了以下观点:当术中潜在损伤及随之而来的神经功能缺损风险较高时,可避免髓母细胞瘤的全切。

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