Lassaletta Alvaro, Perez-Olleros Pilar, Scaglione Cristina, Sirvent Sara, De Prada Inma, Perez-Martinez Antonio, Ruiz-Hernandez Alberto, Madero Luis
Department of Pediatric Hematology-Oncology, Hospital Niño Jesús, Avda, Menendez Pelayo 65, Madrid 28009, Spain.
J Neurooncol. 2007 Jul;83(3):303-6. doi: 10.1007/s11060-006-9327-6. Epub 2007 Jan 24.
Ependymoma is the third most common CNS tumor in children. Neuraxis dissemination at the time of diagnosis is rare and occurs in fewer than 10% of patients. Recent advances in neuroimaging, neurosurgery, and radiation therapy have improved disease control and functional outcomes for children with ependymoma. However, children under the age of 3 years with ependymoma and leptomeningeal spread historically have had worse outcomes. It is not clear if age alone, or a combination of risk factors such us unfavorable location, which may prevent gross total resection, and withholding radiation therapy may have contributed to poor outcomes in younger age groups. The reason for attempting GTR is based on results from retrospective and prospective studies, where the most important prognostic factor is extent of resection. These patients are not candidates for craniospinal irradiation. Therefore, new therapeutic approaches must be attempted. This is a case report of a posterior fossa ependymoma with leptomeningeal dissemination in a two-year-old child, successfully treated with dose intensive chemotherapy and intrathecal liposomal cytarabine.
室管膜瘤是儿童中第三常见的中枢神经系统肿瘤。诊断时神经轴扩散罕见,发生率不到10%的患者。神经影像学、神经外科和放射治疗的最新进展改善了室管膜瘤患儿的疾病控制和功能预后。然而,3岁以下患有室管膜瘤和软脑膜播散的儿童历来预后较差。目前尚不清楚是年龄本身,还是诸如不利位置(可能妨碍肿瘤全切)等危险因素的组合以及不进行放射治疗是否导致了较年轻年龄组的不良预后。尝试进行肿瘤全切的原因基于回顾性和前瞻性研究的结果,其中最重要的预后因素是切除范围。这些患者不适合进行全脑全脊髓照射。因此,必须尝试新的治疗方法。本文报告了一例两岁儿童后颅窝室管膜瘤伴软脑膜播散,经剂量密集化疗和鞘内注射脂质体阿糖胞苷成功治疗的病例。