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儿童视交叉胶质瘤。对视交叉胶质瘤自然病史及颅脑放疗疗效的重新评估。

Chiasmatic gliomas of childhood. A reappraisal of natural history and effectiveness of cranial irradiation.

作者信息

Packer R J, Savino P J, Bilaniuk L T, Zimmerman R A, Schatz N J, Rosenstock J G, Nelson D S, Jarrett P D, Bruce D A, Schut L

出版信息

Childs Brain. 1983;10(6):393-403.

PMID:6661937
Abstract

Disagreement exists concerning the natural history and treatment of chiasmatic gliomas (CG) of childhood. We reviewed our experience in 21 cases of surgically verified CG with a median age of 4 years at time of diagnosis, followed for a median of 5.3 years (range 3-14.8 years). Initial treatment included radiation therapy (RT) in 18 patients, chemotherapy in 2, and observation in 1. Disease recurrence, defined as progressive visual or neurological deterioration, was documented in 10 children (48%), occurring at a median of 6 years after diagnosis. 5-year actuarial survival was 89%, but fell to 60% by 10 years. Visual improvement after RT was uncommon, occurring once. Intellectual deficits were noted in 5 of 17 survivors. We compared our results to that of other patients reported and concluded that: (1) CG may act aggressively independent of their location in the visual pathway at time of diagnosis; (2) the beneficial effects of RT are difficult to document; (3) progressive disease may occur late in the course of illness, and (4) intellectual sequelae are common in long-term survivors.

摘要

关于儿童视交叉胶质瘤(CG)的自然病史和治疗存在分歧。我们回顾了21例经手术证实的CG患者的经验,诊断时的中位年龄为4岁,中位随访时间为5.3年(范围3 - 14.8年)。初始治疗包括18例患者接受放射治疗(RT),2例接受化疗,1例接受观察。10名儿童(48%)出现疾病复发,定义为进行性视力或神经功能恶化,复发中位时间为诊断后6年。5年实际生存率为89%,但到10年时降至60%。RT后视力改善罕见,仅出现过一次。17名幸存者中有5名存在智力缺陷。我们将我们的结果与其他报告的患者结果进行了比较,并得出结论:(1)CG在诊断时可能独立于其在视觉通路中的位置而具有侵袭性;(2)RT的有益效果难以证明;(3)疾病进展可能在病程后期发生;(4)智力后遗症在长期幸存者中很常见。

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