颅内生殖细胞肿瘤的初次化疗：第三次国际中枢神经系统生殖细胞肿瘤研究结果。

Primary chemotherapy for intracranial germ cell tumors: results of the third international CNS germ cell tumor study.

机构信息

Department of Pediatric Oncology, IOP-GRAACC/UNIFESP, São Paulo, SP, Brazil.

出版信息

Pediatr Blood Cancer. 2010 Mar;54(3):377-83. doi: 10.1002/pbc.22381.

DOI:10.1002/pbc.22381

PMID:20063410

Abstract

BACKGROUND

The treatment of central nervous system (CNS) germ cell tumors (GCT) remains controversial. The purpose of this study was to demonstrate efficacy of a chemotherapy only strategy, with less morbidity, when compared to regimens with irradiation.

METHODS

Between January 2001 and December 2004 newly diagnosed patients with CNS GCT were treated with one of two risk-tailored chemotherapy regimens. Twenty-five patients aged 4 months to 24.5 years were stratified: Regimen A consisted of 4-6 cycles of carboplatin/etoposide alternating with cyclophosphamide/etoposide for low risk (LR) localized germinoma with normal cerebrospinal fluid (CSF) and serum tumor markers. Regimen B consisted of 4-6 cycles of carboplatin/cyclophosphamide/etoposide for intermediate-risk (IR) germinoma with positive human chorionic gonadotrophin-beta (HCGbeta) and/or CSF HCGbeta <50 mIU/ml and high-risk (HR) biopsy-proven non-germinomatous malignant elements (MMGCT) or elevated serum/CSF alpha-fetoprotein and/or HCGbeta serum/CSF >50 mIU/ml.

RESULTS

Eleven patients were classified as LR, 2 IR, and 12 HR. Seventeen (68%) patients achieved complete radiographic and marker responses after two courses and 19 (76%) after four courses of chemotherapy. Eleven patients relapsed at a mean of 30.8 months; eight of them subsequently received irradiation. The 6-year event free and overall survival for the 25 patients was 45.6% and 75.3%, respectively.

CONCLUSION

These intensive chemotherapy regimens proved less effective than irradiation containing regimens. Our results indicate that, at the present time, standard treatment for CNS GCT continues to include irradiation either alone or combined with chemotherapy for pure germinomas and with chemotherapy for those with MMGCT.

摘要

背景

中枢神经系统（CNS）生殖细胞瘤（GCT）的治疗仍存在争议。本研究旨在证明与含放疗的方案相比，采用化疗方案具有较少的发病率，且疗效相当。

方法

2001 年 1 月至 2004 年 12 月，新诊断的 CNS GCT 患者接受了两种风险调整化疗方案中的一种治疗。25 名年龄在 4 个月至 24.5 岁的患者进行了分层：方案 A 由 4-6 个周期的卡铂/依托泊苷与环磷酰胺/依托泊苷交替组成，用于局部低危（LR）精原细胞瘤，伴正常脑脊液（CSF）和血清肿瘤标志物。方案 B 由 4-6 个周期的卡铂/环磷酰胺/依托泊苷组成，用于中危（IR）精原细胞瘤，伴人绒毛膜促性腺激素-β（HCGβ）阳性和/或 CSF HCGβ<50mIU/ml 和高危（HR）活检证实的非生殖细胞瘤恶性成分（MMGCT）或升高的血清/CSF 甲胎蛋白和/或 HCGβ血清/CSF>50mIU/ml。

结果

11 例患者被归类为 LR，2 例 IR，12 例 HR。17 例（68%）患者在两个疗程后和 19 例（76%）患者在四个疗程的化疗后达到完全的影像学和标志物反应。17 例患者在平均 30.8 个月时复发，其中 8 例随后接受了放疗。25 例患者的 6 年无事件生存率和总生存率分别为 45.6%和 75.3%。