Optic pathway hypothalamic gliomas in children under three years of age: The role of chemotherapy
Optic pathway hypothalamic gliomas in children under three years of age: The role of chemotherapy
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DOI：
10.1159/000028996
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发表时间：
2000-09-01
影响因子：
0.7
通讯作者：
Tomita, T
中科院分区：
文献类型：
--
作者：
Silva, MM;Goldman, S;Tomita, T
目的：视路/下丘脑胶质瘤（Optic pathway/hypothalamic gliomas，OPHGs）好发于幼儿，治疗方法包括手术切除、放疗（radiation therapy，RT）和化疗。OPHG在3岁以下幼儿中的化学敏感性尚未得到很好的记录。我们分析了14例接受化疗伴或不伴手术的患者。材料和方法：1988年至1998年，14名年龄小于3岁（中位年龄为10个月）的OPHG儿童接受了治疗。所有病例均进行了磁共振成像。脑积水8例，间脑综合征6例。其中只有3例有神经纤维瘤病-l的证据。5例肿瘤部分切除，4例脑室腹腔分流术时行内镜活检。病理检查显示5例为低度恶性星形细胞瘤，4例为幼年型毛细胞星形细胞瘤。所有患者均接受化疗：8例接受卡铂治疗，4例接受卡铂和长春新碱联合治疗，2例接受其他药物联合治疗。结果：14例患者中有8例（57%）在15个月至8年的随访时间内肿瘤持续缩小。5年无进展生存率为63%。这些肿瘤减少通常伴随着临床改善。间脑综合征6例中4例对单独化疗有效。然而，其他5例患者出现疾病进展; 3例在治疗期间，2例在治疗后（分别为9个月和2年）。这5例患者在化疗前均行肿瘤部分切除术。结论：大多数OPHG对化疗有反应。由于这些肿瘤的进展缓慢和其他治疗方式的不良反应，我们建议化疗作为OPHG的主要治疗方法。我们目前的数据表明，部分手术切除并不能提高婴儿或3岁以下儿童OPHG的化疗效果。版权所有（C）2000 S. Karger AG，巴塞尔。
Objectives: Optic pathway/hypothalamic gliomas (OPHGs) tend to occur in young children, Treatment options consist of surgical resection, radiation therapy (RT) and chemotherapy, Due to complications induced by surgery and RT, chemotherapy has gained significant recognition for the treatment of OPHG in young children. Chemosensitivity of OPHG in very young children under 3 years of age has not been well documented. We analyzed 14 patients who were treated with chemotherapy with or without surgery. Materials and Methods: Fourteen children younger than 3 years (median age of 10 months) with OPHG were treated between 1988 and 1998. Magnetic resonance imaging was obtained in all cases. Hydrocephalus was present in 8 patients and diencephalic syndrome was noted in 6. Only 3 of these had evidence of neurofibromatosis-l. Five patients had partial tumor resection and 4 had endoscopic biopsy at the time of ventriculoperitoneal shunt placement, Pathological examination revealed low-grade astrocytoma in 5 and juvenile pilocytic astrocytoma in 4. All patients received chemotherapy: carboplatin in 8, a combination of carboplatin and vincristine in 4 and a combination of other agents in 2. Results: Eight (57%) of 14 patients had a sustained reduction of tumor during the follow-up time between 15 months and 8 years. The 5-year progression free survival was 63%. These tumor reductions were often accompanied by clinical improvements. Diencephalic syndrome responded to chemotherapy alone in 4 of 6 patients. However, 5 others had progressive disease; 3 during the treatment and 2 following the treatment (9 months and 2 years, respectively). All these 5 patients had a partial tumor resection prior to chemotherapy. Conclusion: A majority of OPHGs responds to chemotherapy. Due to slow progression of these tumors and adverse effects of other therapeutic modalities, we recommend chemotherapy as a primary treatment for OPHGs. Our present data indicates that partial surgical resection does not enhance chemotherapy effectiveness for OPHGs in infants or children younger than 3 years. Copyright(C) 2000 S. Karger AG, Basel.