Division of Hematology/Oncology, Children's Hospital of Fudan University, Shanghai, China.
Pediatr Blood Cancer. 2011 Dec 15;57(7):1113-6. doi: 10.1002/pbc.23002. Epub 2011 Jun 16.
To present the characteristics and treatment outcome of patients with intraocular retinoblastoma in a Chinese cooperative group.
Between January 2005 and March 2009, 159 eyes of 133 patients with retinoblastoma were included in this retrospective study. The International Classification of Retinoblastoma (ICRB) staging system was noted for each patient. Cases with visible extraocular extension at diagnosis were excluded. The patient data were reviewed for demographic information, clinical findings, and managements.
Of 133 cases, there were 83 (62%) male and 50 (38%) female, ranging in age from 2 months to 134 months (median, 23 months; mean, 26 months). There were 26 bilateral cases (20%). One hundred and twenty-four cases (93%) were deemed sporadic and nine cases (7%) were deemed familial. Leukocoria was the most common presenting symptom. One hundred and twenty-three eyes (77%) of 123 patients (92%) had no visual potential. Only 36 eyes (23%) of 30 patients (23%) utilized vision-preserving treatments. The ocular salvage rate was 83% (30/36) for this group. The cumulative probability of survival was 98% (Kaplan-Meier method) at 60 months follow up.
The overall survival rate of this study is in agreement with data from developed countries. In appropriate patients, systemic chemotherapy, and focal ophthalmic therapy are effective and carry little morbidity. Compared with more medically developed countries, there are still many challenges in the management of retinoblastoma in China. Early detection and doctor education should be an important future goal. Pediatr Blood Cancer 2011; 57: 1113-1116. © 2011 Wiley Periodicals, Inc.
介绍中国合作组中眼内视网膜母细胞瘤患者的特征和治疗结果。
2005 年 1 月至 2009 年 3 月期间，对 133 例视网膜母细胞瘤患者的 159 只眼进行了回顾性研究。每位患者均采用国际视网膜母细胞瘤分类（ICRB）分期系统进行分期。排除诊断时可见眼外延伸的病例。对患者的人口统计学信息、临床发现和治疗进行了回顾。
133 例中，男性 83 例（62%），女性 50 例（38%），年龄 2 个月至 134 个月（中位数 23 个月，平均 26 个月）。双侧病变 26 例（20%）。124 例（93%）为散发病例，9 例（7%）为家族性病例。白瞳症是最常见的首发症状。123 例患者（92%）的 123 只眼（77%）无光感。仅 30 例患者（23%）的 36 只眼（23%）采用了保视力治疗。该组眼保存率为 83%（30/36）。60 个月随访时，累积生存率为 98%（Kaplan-Meier 法）。
本研究的总体生存率与发达国家的数据一致。在适当的患者中，全身化疗和局部眼科治疗是有效的，且并发症少。与医疗水平更发达的国家相比，中国在视网膜母细胞瘤的治疗方面仍面临诸多挑战。早期发现和医生教育应是未来的重要目标。儿科血液肿瘤学 2011；57：1113-1116。©2011 Wiley Periodicals，Inc.
