维吾尔族儿童视网膜母细胞瘤的临床特点及预后分析

Analysis of the clinical features and prognosis of Uyghur children retinoblastoma

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 analyze the clinical features and prognosis of Uyghur children retinoblas- toma. Methods The diagnosed age, gender composition, clinical symptoms, clinical staging, family histo- ry and prognosis of 59 cases （62 eyes） of Uighur RB children were analyzed. Results Among all the new- ly diagnosed RB Children ： The average age was （ 2.02± O. 99 ） and there was no statistically significant difference in sex composition（ P 〈 0.05 ）. The main clinical symptoms included leukocoria （71.2%） ,stra- bismus （ 13.6% ）. 9 cases （ 14.5% ） were in clinical stage A - C, 23 cases（37. 1% ） in D stage, 30 ea- ses（48.4% ） in E stage. The pathological grade from class I to class IV were 11 cases （ 18.6% ） ,30 cases （50. 8% ） ,12 eases （20. 3% ） and 6 cases （ 10. 2% ）, respectively. There was no clear family disease his- tory. Among the RB children of clinical stage A - C, 4 eyes received laser or cryotherapy, 3 eyes were enu- cleated and other 2 eyes were given up, thus the preservation rate was 44. 4%. Two cases of RB children of clinical stage D were enucleated after conservative treatment and no tumor proliferation was found in other 21 eyes, thus the retention rate was 91.3%. 12 cases of RB children of clinical stage E were enucleated and the eye retention rate was 60. 0%. 3 eases of RB children gave up treatment or were missed in the follow- up. As for the other 56 cases, the follow-up showed that 3 eases were died （5 eyes） （5.4%）, and the o- verall survival rate was 94. 6%. Conclusion The Uyghur children＇s RB occurs at younger age, and is hard to diagnose. Early screening,diagnosis and comprehensive treatment can help to improve the RB children＇s survival rate and eye preservation rate.