摘要目的:观察双眼视网膜母细胞瘤（RB）不同治疗方法与高危病理因素（HHF）的关系。方法:回顾性系列病例研究。1999年至2018年于北京大学人民医院眼科经病理学检查确诊的双眼RB患儿73例73只眼纳入研究。其中，男性50例（68.5%，50/73），男性23例（31.5%，23/73 ）；有家族遗传史11例。首次确诊年龄（14.8±15.6）个月；首次干预时间（3.97±4.74）个月。按照眼内RB国际分期分级标准，73只眼中分期为C、D、E期者分别为2 （2.7%，2/73）、15 （20.5%，15/73）、56 （76.7%，56/73）只眼。全身麻醉下应用广角数码儿童视网膜成像系统行双眼检查。根据临床表现及影像学检查结果首次治疗行眼球摘除或保眼治疗，包括全身化学药物治疗（以下简称为化疗）联合眼动脉内化疗（IAC ）、玻璃体腔注射化疗药物、冷冻、激光光凝、经瞳孔温热疗法、放射治疗等局部治疗。保眼治疗失败后行眼球摘除手术。摘除的眼球标本制作成切片，眼病理医生阅片并评判是否存在HHF。变量间比较采用 t检验；不同治疗方式的HHF发生率及其他分类变量比较采用 χ2检验。 结果:73只眼中，首次治疗为眼球摘除者21只眼（28.8%，21/73 ）；保眼治疗者52只眼（71.2%，52/73 ）。眼球摘除后，复发转移9例（12.3%，9/73），其中死亡7例（9.6%，7/73）。有复发转移者、无复发转移者干预时间分别为（7.4±7.3）、（3.5±4.1 ）个月；复发转移者首次干预时间显著晚于无复发转移者，但差异无统计学意义（ t=-1.561， P=0.154 ）。病理学检查结果提示，存在HHF者26只眼（35.6%，26/73 ），其中D、E期分别为4 （26.7%，15/26）、22 （39.3%，22/56）只眼。眼球摘除前接受其他治疗者，眼球摘除后HHF发生率均低于未接受相应治疗者，但差异无统计学意义（ χ2=1.852、0.074、0.000、1.007、0.007、2.729， P>0.05）。26只眼中，全身化疗联合、未联合IAC分别为5 （83.3%，5/6 ）、21 （31.3%，21/67）只眼，HHF发生率差异有统计学意义（ χ2=4.422， P=0.035）。 结论:眼球摘除前接受IAC保眼治疗对HHF有明显影响。

abstractsObjective:To evaluate the pathological features of bilateral retinoblastoma (RB) and the relationship between different treatments and high-risk histopathologic features (HHF).Methods:Retrospective series of case studies. From 1999 to 2018, 73 patients with binocular RB diagnosed by pathological examination in Department of Ophthalmology, Peking University People's Hospital were included in the study. Among them, 50 patients were male (68.5%, 50/73), 23 patients were females (31.5%, 23/73); 11 patitents had a family history of RB. The mean age at the first diagnosis was 14.8±15.6 months. The average time between first diagnosis and first intervention was 3.97±4.74 months. According to the international classification standard of intraocular RB staging, among the 73 eyes, C, D and E stages were 2 (2.7%, 2/73), 15 (20.5%, 15/73), and 56 (76.7%, 56/73) eyes, respectively. Ocular images for each patient were obtained using a wide-angle contact fundus camera during examination under general anaesthesia. The treatment protocol (globe salvaging or enucleation) depended on the result of several clinical features. Globe salvaging treatment included chemotherapy combined with local therapy such as intra-arterial chemotherapy (IAC), intravitreal chemotherapeutics injection, cryotherapy, laser, transpupillary thermotherapy and radiotherapy. If globe salvaging failed, enucleation was offered and histopathologic analysis was conducted of the enucleated eye, the ophthalmic pathologist read and evaluated the presence of HHF. Independent samples t-test was performed to compare the continuous variables. The pathological features and the relationship between different treatments and HHF were analyzed. Group difference was calculated with chi-square. Results:Among the 73 eyes, the first treatment was enucleation in 21 eyes (28.8%, 21/73); 52 eyes (71.2%, 52/73) were treated with eye protection. After enucleation, 9 cases (12.3%, 9/73) had recurrence and metastasis, and 7 cases (9.6%, 7/73) died. The intervention time of patients with recurrence and metastasis and those without recurrence and metastasis were 7.4±7.3 and 3.5±4.1 months respectively; the first intervention time of patients with recurrence and metastasis was significantly later than that of patients without recurrence and metastasis, but the difference was not statistically significant ( t=-1.561, P=0.154). The pathological examination results showed that there were 26 eyes (35.6%, 26/73) with HHF, 4 (26.7%, 15/26) and 22 (39.3%, 22/56) eyes were in stage D and E, respectively. Those who received other treatments before enucleation had lower HHF percentages after enucleation than those who did not receive corresponding treatments, but the difference was not statistically significant ( χ2=1.852, 0.074, 0.000, 1.007, 0.007, 2.729; P>0.05). Among the 26 eyes, 5 (83.3%, 5/6) and 21 (31.3%, 21/67) eyes were treated with systemic chemotherapy combined with and without IAC, respectively, and there was a significant difference in the percentage of HHF ( χ2=4.422, P=0.035). Conclusions:IAC eye-preserving therapy before enucleation has a significant effect on HHF.