双眼视网膜母细胞瘤46例的临床特点及疗效

Clinical characteristics and therapeutic effect of 46 cases of bilateral retinoblastoma

目的:分析46例双眼视网膜母细胞瘤(retinoblastoma,RB)的临床特点及治疗效果。方法:回顾性分析2008年12月至2019年12月重庆市陆军军医大学陆军特色医学中心收治的46例接受静脉化疗联合经瞳孔温热疗法(transpupillary thermotherapy,TTT)或眼摘治疗的双眼RB住院患儿的临床资料,对患儿的保眼率、摘眼率、视力情况及化疗不良反应进行评估。结果:46例患儿中,男27例,女19例,初诊年龄为(13.21±11.13)个月。单纯化疗10例,化疗+TTT治疗11例,化疗+TTT+眼摘治疗17例,化疗+冷凝治疗2例,化疗+冷凝+眼摘治疗6例。46例92眼总保眼率73.1%(57/78),残留视力眼占64.1%(50/78),各期保眼率:A、B期均100.0%,C期86.7%,D期94.1%,E期35.7%。手术摘除24眼,总摘眼率26.1%(24/92),E期手术摘除21眼,占E期患眼60.0%(21/35)。平均化疗(4.1±1.9)次,化疗的骨髓抑制主要表现为白细胞减少、血小板减少及血红蛋白减少。46例患儿随访时间(35.4±23.8)个月,死亡7例,总病死率15.2%(7/46);存活39例,总存活率为84.8%(39/46),5年累积生存率为80.2%。结论:静脉化疗联合局部治疗总体疗效较好,在双眼RB患儿治疗中占据重要地位。化疗具有骨髓抑制作用,停止化疗后骨髓抑制逐渐恢复。

Objective:To analyze the clinical characteristics and therapeutic effect of 46 patients with bilateral retinoblastoma (RB).Methods:The clinical data of 46 patients with bilateral retinoblastoma who received intravenous chemotherapy combined with transpupillary thermotherapy (TTT) or enucleation from December 2008 to December 2019 in our department were analyzed retrospectively.The eye salvage rate,enucleation rate,visual acuity,and chemotherapy side effects were evaluated.Results:The 46 enrolled patients were 27 males and 19 females,at an average age of (13.21±11.13) months at the first visit.Among them,10 received chemotherapy,11 received chemotherapy combined with TTT,17 received chemotherapy combined with TTT and enucleation,and 2 received chemotherapy combined with freezing,6 received chemotherapy combined with freezing and enucleation.After treatment,58 eyes were salvaged,with a total salvage rate of 73.1% (57/78),and the eyes that preserved vision account for 64.1% (50/78).The eye salvage rate in each stage were 100.0% for stage A and B,86.7% for stage C,and 94.1% for stage D,35.7% for stage E.Twenty-four eyes were enucleated,with a total enucleation rate of 26.1% (24/92),and among 35 eyes at stage E,21 eyes were enucleated,accounting for 60.0% (21/35).The average time of chemotherapy was 4.1±1.9 and the myelosuppressive effects of chemotherapy include leucopenia,thrombocytopenia and hemoglobinopenia.During the mean follow-up time of(35.4±23.8) months,7 (15.2%) patients died,and 39 (84.8%) cases survived.The 5-year cumulative survival rate was 80.2%.Conclusion:Intravenous chemotherapy combined with local treatment has a good overall effect and plays an important role in the treatment of bilateral retinoblastoma.Intravenous chemotherapy leads to myelosuppression,and the myelosuppression gradually recovered after stopping intravenous chemotherapy.