Prospective neuraxis MRI surveillance reveals a high risk of leptomeningeal dissemination in diffuse intrinsic pontine glioma
Prospective neuraxis MRI surveillance reveals a high risk of leptomeningeal dissemination in diffuse intrinsic pontine glioma
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DOI：
10.1007/s11060-010-0301-y
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发表时间：
2011-03-01
影响因子：
3.9
通讯作者：
Narayana, Ashwatha
中科院分区：
文献类型：
--
作者：
Sethi, Rajni;Allen, Jeffrey;Narayana, Ashwatha
弥漫性脑桥内胶质瘤（DIPG）的预后仍然较差。失败主要是局部的，在前MRI时代系列中，4 - 33%的患者发生软脑膜播散（LD）。初步治疗后常规颅脊髓成像可能揭示其他复发模式复发。回顾性分析了2006年至2009年期间接受DIPG治疗的16例连续儿科患者。记录治疗方案、复发模式、生存率和病理诊断。14例患者接受了累及野放疗至54戈伊，2例患者在就诊时接受了LD的颅脊髓放疗。在诊断时和放疗后4个月间隔进行神经轴MRI检查。15例患者出现疾病进展（中位无进展生存期5.0 +/-A 1.2个月），13例患者死亡（中位生存期9.0 +/-A 1.4个月）。局部失败12例（75%）。9例患者（56%）发生LD。LD在诊断时存在于3例患者中，在初始分期和治疗后存在于6例患者中，在尸检期间存在于2例患者中。中位总生存期为12.0 +/-A 3.3个月（无LD）和8.0 +/-A 2.1个月（有LD）（P = 0.059，对数秩检验）。无LD组的中位无进展生存期为9.5 +/-A 3.9个月，LD组为3.0 +/-A 2.1个月（P = 0.012，对数秩检验）。LD的高发病率可能反映了脊柱MRI监测的广泛使用。所有患者应在诊断和随访时进行常规颅脊髓成像。在未来的临床试验中应考虑中枢神经系统预防。
Prognosis of diffuse intrinsic pontine gliomas (DIPGs) remains poor. Failure has been predominantly local, with leptomeningeal dissemination (LD) occurring in 4-33% of patients in pre-MRI era series. Routine craniospinal imaging after initial treatment may reveal other relapse patterns relapse. Sixteen consecutive pediatric patients with DIPG treated between 2006 and 2009 were retrospectively reviewed. Treatment regimens, recurrence patterns, survival, and pathologic diagnosis were recorded. Fourteen patients received involved-field radiotherapy to 54 Gy, and two patients received craniospinal irradiation for LD at presentation. Neuraxis MRI was performed at diagnosis and at 4 month intervals following radiotherapy. Fifteen patients have had progression of disease (median progression-free survival 5.0 +/- A 1.2 months), and 13 patients have died (median survival 9.0 +/- A 1.4 months). Local failure occurred in 12 patients (75%). LD occurred in nine patients (56%). LD was present at diagnosis in three patients, after initial staging and treatment in six patients, and during autopsy in two patients. Median overall survival was 12.0 +/- A 3.3 months without LD and 8.0 +/- A 2.1 months with LD (P = 0.059, log rank test). Median progression-free survival was 9.5 +/- A 3.9 months without LD and 3.0 +/- A 2.1 months with LD (P = 0.012, log rank test). The high incidence of LD probably reflects liberal use of spine MRI surveillance. All patients should undergo routine craniospinal imaging at diagnosis and follow-up. Central nervous system prophylaxis should be considered in future clinical trials.