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目的回顾性分析维吾尔族儿童视网膜母细胞瘤(RB)的临床特点及预后。方法对59例(62只眼)维吾尔族RB患儿的初诊年龄分布及性别构成、临床症状、临床分期、家族史及治疗预后等资料进行分析。结果初诊患儿年龄为(2.02±0.99)岁,患儿性别构成比的差异无统计学意义(P>0.05)。临床症状主要包括白瞳症(71.2%)和斜视(13.6%)。临床分期A~C期9只眼(14.5%),D期23只眼(37.1%),E期30只眼(48.4%);病理分级I级11例(18.6%),II级30例(50.8%),III级12例(20.3%),IV级6例(10.2%),未见明确家族史。A~C期患儿中,4只眼接受激光或冷冻治疗保存眼球,3只眼摘除眼球,2只眼放弃治疗,眼球保存率为44.4%;D期患儿经保守治疗后眼球摘除2只,21只眼未见肿瘤扩散,患儿视力恢复正常,眼球保存率为91.3%;E期30只眼,12只眼行眼球摘除术治疗,眼球保存率为60.0%。59例患儿中,失访或放弃治疗者3例(5只眼),死亡3例(5只眼),总存活率为94.6%。结论维吾尔族儿童RB发病年龄较小且起病较为隐匿,早期接受筛查、诊断及综合治疗将有助于提高RB患儿生存率及眼球保存率。
Objective To retrospectively analyze the clinical features and prognosis of Uygur children with retinoblastoma (RB). Methods The age at diagnosis, gender composition, clinical symptoms, clinical stage, family history and prognosis of RB children with Uygur from 59 cases (62 eyes) were analyzed. Results The age of newly diagnosed children was (2.02 ± 0.99) years old, and there was no significant difference in sex composition ratio (P> 0.05). Clinical symptoms include white pupil disease (71.2%) and strabismus (13.6%). There were 9 eyes (14.5%) in stage A to C, 23 eyes (37.1%) in stage D, 30 eyes (48.4%) in stage E, 11 cases (18.6% 50.8%), grade III in 12 cases (20.3%), grade IV in 6 cases (10.2%), no clear family history. Among the children with A ~ C stage, 4 eyes underwent laser or cryotherapy to preserve the eye, 3 eyes to remove the eyeball and 2 eyes to abandon the treatment, the eyeball preservation rate was 44.4%. In the D group, 2 , No tumor spread in 21 eyes, normal vision recovery in children, the eyeball preservation rate was 91.3%; E period of 30 eyes, 12 eyes underwent enucleation treatment, the eyeball preservation rate of 60.0%. Among the 59 children, 3 (5 eyes) lost or abandoned the treatment and 3 died (5 eyes). The overall survival rate was 94.6%. Conclusion The onset age of RB in Uygur children is relatively small. Early screening, diagnosis and comprehensive treatment will help to improve the survival rate and eyeball preservation rate in children with RB.