Fabian Ido D, Stacey Andrew W, Johnson Kenneth P, Onadim Zerrin, Chowdhury Tanzina, Duncan Catriona, Reddy M Ashwin, Sagoo Mandeep S
Ocular Oncology Service, Moorfields Eye Hospital NHS Foundation Trust, London, UK.
Retinoblastoma Service, Royal London Hospital, London, UK.
Br J Ophthalmol. 2017 Jan;101(1):82-88. doi: 10.1136/bjophthalmol-2016-309710. Epub 2016 Dec 13.
Eye salvage rate for group D retinoblastoma using intravenous chemotherapy (IVC) as a primary modality is <50%. To report on 13 years' experience with the use of primary IVC for group D retinoblastoma.
A retrospective analysis of 64 group D eyes (52 patients) treated with primary IVC, from 2002 to 2014.
The median age at presentation was 11.0 months (mean: 18.6, range: 0.6-144.0), 35 (67%) patients had bilateral disease, 38 (73%) germline disease and 8 (15%) cases were familial. In addition to IVC, patients received a median number of three treatments (mean: 6, range: 0-24), including thermotherapy/cryotherapy, plaque radiotherapy, intra-ophthalmic artery chemotherapy (IAC) and/or intravitreous chemotherapy. External beam radiotherapy (EBRT) was used in five eyes, all of which were eventually enucleated. In a median follow-up time of 55 months (mean: 64, range: 14-156), 63% of eyes were salvaged. By the Kaplan-Meier survival analysis, globe salvage rate was 83%, 70%, 59% and 45% at 1, 3, 5 and 10 years, respectively. There were no cases of metastatic spread from intraocular retinoblastoma and no deaths. IVC-related adverse events included febrile neutropenia in 21 (40%) patients and anaphylactic reaction to carboplatin in 2 (4%), all conservatively resolved. Of the patients receiving IAC, third and sixth nerve palsies were documented in two (10%) and one (5%) eyes, respectively.
Primary IVC for group D eyes, with adjuvant treatments as required, was found to be a safe and efficient approach, achieving 63% eye salvage rate, no metastatic spread from intraocular retinoblastoma and no deaths. IAC has now replaced EBRT as a successful salvage treatment.
以静脉化疗（IVC）作为主要治疗方式时，D 组视网膜母细胞瘤的眼球挽救率低于 50%。报告 13 年来使用原发性 IVC 治疗 D 组视网膜母细胞瘤的经验。
对 2002 年至 2014 年期间接受原发性 IVC 治疗的 64 只 D 组患眼（52 例患者）进行回顾性分析。
初诊时的中位年龄为 11.0 个月（平均：18.6 个月，范围：0.6 - 144.0 个月），35 例（67%）患者为双眼患病，38 例（73%）为遗传性疾病，8 例（15%）为家族性病例。除 IVC 外，患者接受的治疗次数中位数为 3 次（平均：6 次，范围：0 - 24 次），包括热疗/冷冻疗法、敷贴放射疗法、眼内动脉化疗（IAC）和/或玻璃体内化疗。5 只眼使用了外照射放疗（EBRT），所有这些眼最终均被摘除。中位随访时间为 55 个月（平均：64 个月，范围：14 - 156 个月），63%的患眼得以挽救。通过 Kaplan-Meier 生存分析，1 年、3 年、5 年和 10 年时的眼球挽救率分别为 83%、70%、59%和 45%。未出现眼内视网膜母细胞瘤转移扩散的病例，也无死亡病例。与 IVC 相关的不良事件包括 21 例（40%）患者出现发热性中性粒细胞减少，2 例（4%）患者对卡铂发生过敏反应，均经保守治疗缓解。在接受 IAC 的患者中，分别有 2 只眼（10%）和 1 只眼（5%）记录到动眼神经和展神经麻痹。
对于 D 组患眼，采用原发性 IVC 并根据需要进行辅助治疗，是一种安全有效的方法，眼球挽救率达到 63%，未出现眼内视网膜母细胞瘤转移扩散，也无死亡病例。IAC 现已取代 EBRT 成为一种成功的挽救治疗方法。
