Hu Huimin, Zhang Weiling, Wang Yizhuo, Huang Dongsheng, Shi Jitong, Li Bin, Zhang Yi, Zhou Yan
Department of Pediatrics, Beijing Tongren Hospital, West South road 2, Yizhuang Economic and Technological Development Zone, Daxing District, Beijing, 100176, China.
Department of Ophthalmology, Beijing Tongren Hospital, Capital Medical University, Beijing, 100176, China.
BMC Ophthalmol. 2018 Apr 23;18(1):107. doi: 10.1186/s12886-018-0772-8.
Retinoblastoma is the most common primary intraocular tumor and more and more attention has been paid to the developing countries. This study was aimed to evaluate the clinical features, treatment, and prognosis of retinoblastoma patients with central nervous system (CNS) metastasis in Beijing Tongren Hospital, one of the largest tertiary eye centers in China.
Clinical data of 31 consecutive retinoblastoma patients with CNS metastases, who were diagnosed at the Department of Pediatrics in Beijing Tongren Hospital between September 2005 and December 2015, were retrospective analyzed.
The median age at presentation was 29 months (range from 5 to 108 months). Magnetic resonance imaging (MRI) results indicated that 16 patients (56.6%, 16/31) presented with meningeal involvement, 12 (38.7%, 12/31) presented with intracranial mass, 11 (35.5%, 11/31) presented with thickened optic nerve, and 5 (16.1%, 5/31) presented with concurrent meningeal and spinal cord membrane involvement. Retinoblastoma cells were detected in the cerebrospinal fluid (CSF) of 12 patients (44.4%, 12/27). Laboratory examinations on the blood and CSF were performed for 11 patients who had received six cycles of systemic chemotherapy, indicated that the serum level of neurone-specific enolase (NSE) after chemotherapy was significantly lower than that before chemotherapy (P < 0.05). At the end of the follow-up, 25 patients were dead with a median survival time of 6 months (1 d - 21 months), and 6 cases were alive and continued to receive treatment.
Our results were basically consistent with previous reports in the developing countries, and it could be guidance for clinical treatment, prognosis and prevention of CNS metastases in retinoblastoma.
视网膜母细胞瘤是最常见的原发性眼内肿瘤，越来越受到发展中国家的关注。本研究旨在评估中国最大的三级眼科中心之一北京同仁医院视网膜母细胞瘤中枢神经系统（CNS）转移患者的临床特征、治疗及预后。
回顾性分析2005年9月至2015年12月在北京同仁医院儿科确诊的31例连续性视网膜母细胞瘤中枢神经系统转移患者的临床资料。
就诊时的中位年龄为29个月（范围5至108个月）。磁共振成像（MRI）结果显示，16例患者（56.6%，16/31）有脑膜受累，12例（38.7%，12/31）有颅内肿块，11例（35.5%，11/31）有视神经增粗，5例（16.1%，5/31）有脑膜和脊髓膜同时受累。12例患者（44.4%，12/27）的脑脊液（CSF）中检测到视网膜母细胞瘤细胞。对接受六个周期全身化疗的11例患者进行血液和脑脊液实验室检查，结果显示化疗后神经元特异性烯醇化酶（NSE）血清水平显著低于化疗前（P＜0.05）。随访结束时，25例患者死亡，中位生存时间为6个月（1天至21个月），6例存活并继续接受治疗。
我们的结果与发展中国家先前的报道基本一致，可为视网膜母细胞瘤中枢神经系统转移的临床治疗、预后及预防提供指导。
