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儿童颅咽管瘤

Craniopharyngiomas in children.

作者信息

Ohmori Kazumi, Collins John, Fukushima Takanori

机构信息

Department of Neurosurgery, West Virginia University, Morgantown, W.VA., USA.

出版信息

Pediatr Neurosurg. 2007;43(4):265-78. doi: 10.1159/000103306.

Abstract

BACKGROUND

The modern era of pediatric craniopharyngioma treatment includes multiple modalities including microsurgical resection, irradiation, brachytherapy or chemotherapy. No clear consensus as to the best therapeutic approach has yet been established. The aim of this study was to describe the techniques and strategies for the treatment of pediatric craniopharyngiomas in light of a literature review with particular attention to the incidence of adverse postoperative effects.

METHODS

Twenty-seven pediatric patients (median age 9.0 years) who were surgically treated for craniopharyngiomas were evaluated. We reviewed the recent literature for clinical features of craniopharyngiomas in children, including the present cases.

RESULTS

The overall rate of radiographically complete resection of our cases was 92.6%. In the literature we reviewed, the rate of gross total resection was 33-91% (average 57.8%). According to the literature, recurrence rates range from zero to 52.8% (average 16.1%) in the gross total resection group, 51.1% in the subtotal resection without radiation therapy group, and 33.5% in the subtotal resection with radiation therapy group. In six of our most recent cases, for whom we have complete outcome analysis, radiographically complete resection is 100% with only one recurrence so far over 4 years.

CONCLUSIONS

For patients in whom early postoperative MRI reveals complete craniopharyngioma removal, a very low rate of recurrence is anticipated. In the authors' experience, radiographically total excision of even large craniopharyngiomas can be safely achieved by one or a combination of several advanced microsurgical techniques, sometimes by a staged strategy. Major morbidities can be avoided, although moderate morbidities occur and require management.

摘要

背景

儿童颅咽管瘤治疗的现代时期包括多种治疗方式,如显微手术切除、放射治疗、近距离放射治疗或化疗。目前尚未就最佳治疗方法达成明确共识。本研究的目的是根据文献综述描述儿童颅咽管瘤的治疗技术和策略,特别关注术后不良反应的发生率。

方法

对27例接受颅咽管瘤手术治疗的儿童患者(中位年龄9.0岁)进行评估。我们回顾了包括本病例在内的儿童颅咽管瘤的近期文献,以了解其临床特征。

结果

我们病例的影像学完全切除率为92.6%。在我们回顾的文献中,大体全切除率为33%-91%(平均57.8%)。根据文献,大体全切除组的复发率为0%-52.8%(平均16.1%),次全切除未放疗组为51.1%,次全切除加放疗组为33.5%。在我们最近的6例有完整结局分析的病例中,影像学完全切除率为100%,4年多来仅1例复发。

结论

对于术后早期MRI显示颅咽管瘤完全切除的患者,预计复发率非常低。根据作者的经验,即使是大型颅咽管瘤,通过一种或几种先进的显微手术技术,有时采用分期策略,也能安全地实现影像学全切除。虽然会出现中度并发症且需要处理,但可以避免严重并发症。

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