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出生后头六个月的中枢神经系统(CNS)肿瘤:洛杉矶儿童医院的经验,1979 - 2005年

Central nervous system (CNS) tumors in the first six months of life: the Children's Hospital Los Angeles experience, 1979-2005.

作者信息

Serowka K, Chiu Y, Gonzalez I, Gilles F, McComb G, Krieger M, Dhall G, Britt B, Ji L, Sposto R, Finlay J L

机构信息

The Children's Center for Cancer and Blood Diseases, Childrens Hospital Los Angeles, California 90027-6016, USA.

出版信息

Pediatr Hematol Oncol. 2010 Mar;27(2):90-102. doi: 10.3109/08880010903447342.

DOI:10.3109/08880010903447342
PMID:20201690
Abstract

BACKGROUND

The authors report the experience at the Children's Hospital Los Angeles with brain tumors diagnosed before 6 months of age, describing the characteristics of the patients, their tumors, treatment strategies, and prognostic factors.

METHODS

Thirty-three children who were identified between 1979 and 2005 were included. Twelve were female (36%). There were 11 gliomas, 9 choroid plexus tumors, 8 medulloblastomas and supratentorial primitive neuroectodermal tumors (PNET), 2 atypical teratoid/rhabdoid tumors (ATRT), and 1 each of ependymoma, craniopharyngioma, and immature teratoma. Locations of primary tumors included 21 supratentorial (64%) and 7 posterior fossa, and 5 tumors involved both compartments. The treatment strategies included 5 patients with biopsy only, 18 less than gross total resections (<GTRx), and 9 GTRx. Fourteen children (42%) received chemotherapy. Three patients (9%) received irradiation, 1 at initial diagnosis and 2 at relapse. Nine patients (27%) demonstrated metastases, 6 at diagnosis and 3 at relapse.

RESULTS

The Kaplan Meier analysis of event-free survival (EFS) and overall survival (OS) for all patients is 21 +/- 9% and 35 +/- 9% at 5 years. For the glioma patients, the 4-year OS is 48 +/- 17%, while the 5-year OS for the medulloblastoma/PNET/ATRT patients is 12 +/- 11% (p = .39). The 5-year OS for children achieving a GTRx is 64 +/- 21% and for those with <GTRx is 27 +/- 10% (p = .08).

摘要

背景

作者报告了洛杉矶儿童医院对6个月龄前诊断出的脑肿瘤的治疗经验,描述了患者、其肿瘤、治疗策略及预后因素的特征。

方法

纳入了1979年至2005年间确诊的33名儿童。12名女性(36%)。有11例胶质瘤、9例脉络丛肿瘤、8例髓母细胞瘤和幕上原始神经外胚层肿瘤(PNET)、2例非典型畸胎样/横纹肌样肿瘤(ATRT),以及各1例室管膜瘤、颅咽管瘤和未成熟畸胎瘤。原发肿瘤部位包括21例幕上(64%)和7例后颅窝,5例肿瘤累及两个部位。治疗策略包括5例仅行活检的患者、18例次全切除(<GTRx)和9例GTRx。14名儿童(42%)接受了化疗。3例患者(9%)接受了放疗,1例在初诊时接受放疗,2例在复发时接受放疗。9例患者(27%)出现转移,6例在诊断时出现转移,3例在复发时出现转移。

结果

所有患者的无事件生存率(EFS)和总生存率(OS)的Kaplan Meier分析显示,5年时分别为21±9%和35±9%。对于胶质瘤患者,4年总生存率为48±17%,而髓母细胞瘤/PNET/ATRT患者的5年总生存率为12±11%(p = 0.39)。实现GTRx的儿童5年总生存率为64±21%,未达到GTRx的儿童为27±10%(p = 0.08)。

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