Clinical presentation and treatment outcome of retinoblastoma in children of South Western China.
Clinical presentation and treatment outcome of retinoblastoma in children of South Western China.
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DOI：
10.1097/md.0000000000005204
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发表时间：
2016-10
期刊：
影响因子：
1.6
通讯作者：
Chen D
中科院分区：
文献类型：
--
作者：
Gao J;Zeng J;Guo B;He W;Chen J;Lu F;Chen D
研究中国西南地区儿童视网膜母细胞瘤（RB）的临床表现和治疗结果，并确定预后不良的预测因素。对2006 - 2015年华西医院诊断为RB的患儿进行回顾性分析。研究人口学、临床特征及治疗结果。共253例（单侧80.2%，双侧19.8%）。少数民族26例（10.3%）。中位发病年龄为21个月。白斑是最常见的表现（71%）。91.3%为眼内肿瘤，8.7%为眼外肿瘤。眼外RB患者的中位滞后时间长于眼内患者（9个月vs 2个月，P < 0.0001）。眼内组89.5%为晚期D组或E组疾病。眼球摘除是眼内RB的主要治疗方法。然而，在10年的时间里，随着更多的患者接受化疗，去核率不断下降。3年、5年和10年的Kaplan-Meier生存率分别为87.8%、81.4%和74.8%。Cox回归分析显示，眼外RB （P = 0.0008）和治疗放弃（P < 0.0001）与预后不良相关；双侧RB （P = 0.0116）和病理分级pT4 （P = 0.0011）与眼内RB预后不良相关。中国西南地区RB患者多在临床晚期确诊。延迟表现与眼外RB有关，这是预后不良的危险因素。化疗增加了眼部修复，但对总体生存没有影响。对家长和全科医生进行RB早期症状（如白斑）、治疗策略和治疗结果的教育，可以促进RB的早期诊断，提高依从性和预后。
To study the clinical presentation and treatment outcome among children in South Western China with retinoblastoma (RB) and to determine factors predictive of poor outcome. A retrospective review of children diagnosed with RB from 2006 to 2015 at West China Hospital was undertaken. Demographic and clinical characteristics and treatment outcomes were studied. A total of 253 patients (unilateral 80.2%, bilateral 19.8%) were studied. Twenty six patients (10.3%) were from minority ethnic groups of China. The median onset age was 21 months. Leukocoria was the most common presenting sign (71%). Tumors were intraocular in 91.3% cases, extraocular in 8.7% cases. Extraocular RB patients had a longer median lag period than intraocular patients (9 months vs 2 months, P < 0.0001). In the intraocular group, 89.5% were advanced group D or E diseases. Enucleation was the major treatment for intraocular RB. However, over 10 years, the enucleation rate decreased constantly while more patients received chemotherapy. The Kaplan–Meier survival probability was 87.8%, 81.4%, and 74.8% at 3 years, 5 years, and 10 years, respectively. On Cox regression analysis, extraocular RB (P = 0.0008) and treatment abandonment (P < 0.0001) were associated with poor outcome; bilateral RB (P = 0.0116) and advanced pathological grade pT4 (P = 0.0011) were associated with poor outcome of intraocular RB. Most RB patients from South Western China were diagnosed at advanced clinical stage. Delayed presentation is related to extraocular RB which is a risk factor for poor outcome. Chemotherapy increased the eye salvage but had no effects to overall survival. Education for parents and general physicians for the early signs of RB (such as leukocoria), therapeutic strategy and treatment outcomes of RB may promote early diagnosis, improve the compliance, and outcome.