儿童视交叉/下丘脑星形细胞瘤的管理

Management of optic chiasmatic/hypothalamic astrocytomas in children.

作者信息

Steinbok Paul, Hentschel Stephen, Almqvist Per, Cochrane D Douglas, Poskitt Kenneth

机构信息

Department of Surgery, University of British Columbia and British Columbia's Children's Hospital, Vancouver, Canada.

出版信息

Can J Neurol Sci. 2002 May;29(2):132-8.

PMID:12035834

Abstract

OBJECTIVE

The management of optic chiasmatic gliomas is controversial, partly related to failure to separate out those tumors involving the optic chiasm only (chiasmatic tumors) from those also involving the hypothalamus (chiasmatic/hypothalamic tumors). The purpose of this study was: (i) to analyze the outcomes of chiasmatic and chiasmatic/hypothalamic tumors separately; and (ii) to determine the appropriateness of recommending radical surgical resection for the chiasmatic/hypothalamic tumors.

METHODS

A retrospective chart review of all newly diagnosed tumors involving the optic chiasm from 1982-1996 at British Columbia's Children's Hospital was performed.

RESULTS

There were 32 patients less than 16 years of age, 14 with chiasmatic and 18 with chiasmatic/hypothalamic astrocytomas, with an average duration of follow-up of 5.8 years and 6.3 years, respectively. Ten of the patients with chiasmatic tumors and none with chiasmatic/hypothalamic tumors had neurofibromatosis I. Thirteen of the 14 chiasmatic tumors were managed with observation only, and none had progression requiring active intervention. For the chiasmatic/hypothalamic tumors, eight patients had subtotal resections (>95% resection), six had partial resections (50-95%), three had limited resections (<50%), and one had no surgery. There were fewer complications associated with the limited resections, especially with respect to hypothalamic dysfunction. There was no correlation between the extent of resection (subtotal, partial, or limited) and the time to tumor progression (average 18 months).

CONCLUSIONS

In conclusion, chiasmatic and chiasmatic/hypothalamic tumors are different entities, which should be separated out for the purposes of any study. For the chiasmatic/hypothalamic tumors, there was more morbidity and no prolongation of time to progression when radical resections were compared to more limited resections. Therefore, if surgery is performed, it may be appropriate to do a surgical procedure that strives only to provide a tissue diagnosis and to decompress the optic apparatus and/or ventricular system.

摘要

目的

视交叉胶质瘤的治疗存在争议，部分原因是未能将仅累及视交叉的肿瘤（视交叉肿瘤）与那些也累及下丘脑的肿瘤（视交叉/下丘脑肿瘤）区分开来。本研究的目的是：（i）分别分析视交叉肿瘤和视交叉/下丘脑肿瘤的治疗结果；（ii）确定对视交叉/下丘脑肿瘤推荐根治性手术切除的合理性。

方法

对1982年至1996年在不列颠哥伦比亚省儿童医院新诊断的所有累及视交叉的肿瘤进行回顾性病历审查。

结果

有32例年龄小于16岁的患者，14例为视交叉肿瘤，18例为视交叉/下丘脑星形细胞瘤，平均随访时间分别为5.8年和6.3年。14例视交叉肿瘤患者中有10例患有神经纤维瘤病I型，视交叉/下丘脑肿瘤患者中无一例患有该病。14例视交叉肿瘤中有13例仅采用观察治疗，无一例病情进展需要积极干预。对于视交叉/下丘脑肿瘤，8例患者进行了次全切除（切除>95%），6例进行了部分切除（切除50 - 95%），3例进行了有限切除（切除<50%），1例未进行手术。有限切除相关的并发症较少，尤其是下丘脑功能障碍方面。切除范围（次全、部分或有限）与肿瘤进展时间（平均18个月）之间无相关性。