Department of Pediatrics, Beijing Tongren Hospital, Capital Medical University, No. 2, Xihuan South Road, Yizhuang Economic and Technological Development Zone, Beijing, 100176, China.
Department of Ophthalmology Oncology, Beijing Tongren Hospital, Capital Medical University, Beijing, China.
BMC Ophthalmol. 2021 Jan 14;21(1):38. doi: 10.1186/s12886-020-01768-4.
To analyze the clinical characteristics, treatment and prognosis of children with unilateral retinoblastoma (RB) and intracranial segment of retrobulbar optic nerve invasion.
A total of 14 children with unilateral RB and intracranial segment of retrobulbar optic nerve invasion were enrolled in this retrospective study from January 2009 to December 2018. Clinical characteristics, treatment and prognosis were collected and analyzed. Survival curves were calculated by Kaplan-Meier method.
Of 14 cases, there were 7 male and 7 female, ranging in age from 22.85 to 121.97 months (median, 41.03 months). Seventy-one percent of patients came from first-tier cities in China and effected in the left eye. Magnetic resonance imaging (MRI) results indicated that all patients presented with thickened and enhanced optic nerve and intracranial segment of optic nerve invasion. Nine patients received comprehensive therapeutic regimen (chemotherapy, eye enucleation, radiotherapy and intrathecal therapy). The patients were followed up to December 2019, with a median follow-up of 20.6 months. The median disease specific survival was 48.99 ± 8.62 months, and the overall survival (OS) rate was 64.3%. Radiotherapy and comprehensive therapeutic regimen had significant impact on survival time (all p < 0.05).
The overall prognosis of unilateral RB patients with intracranial segment of retrobulbar optic nerve invasion was poor. Chemotherapy and surgical treatment were necessary, but more attention should be paid to radiotherapy and intrathecal therapy for improving prognosis.
分析单侧视网膜母细胞瘤（RB）伴颅内段视神经眶内段侵犯患儿的临床特征、治疗方法和预后。
本回顾性研究纳入了 2009 年 1 月至 2018 年 12 月期间的 14 例单侧 RB 伴颅内段视神经眶内段侵犯患儿。收集并分析了临床特征、治疗方法和预后。采用 Kaplan-Meier 法计算生存曲线。
14 例患儿中，男 7 例，女 7 例；年龄 22.85~121.97 个月，中位数 41.03 个月。71%的患儿来自中国一线城市，左眼受累。磁共振成像（MRI）结果显示所有患儿均存在视神经增粗和强化以及颅内段视神经侵犯。9 例患儿接受了综合治疗方案（化疗、眼球摘除术、放疗和鞘内治疗）。患儿随访至 2019 年 12 月，中位随访时间为 20.6 个月。中位疾病特异性生存时间为 48.99±8.62 个月，总生存率为 64.3%。放疗和综合治疗方案对生存时间有显著影响（均 P<0.05）。
单侧 RB 伴颅内段视神经眶内段侵犯患儿的总体预后较差。化疗和手术治疗是必要的，但为了改善预后，应更加重视放疗和鞘内治疗。
