三侧性视网膜母细胞瘤4例临床分析及文献回顾  

Trilateral retinoblastoma:case series report and literature review

作　　者：顾华丽[1] 王一卓[1] 黄东生[1] 张谊[1] 张伟令[1] 周燕[1] 李楠[1] 高亚楠[1] 任卫山 王群[1] 

机构地区：[1]首都医科大学附属北京同仁医院儿科,北京100176

出　　处：《中国小儿血液与肿瘤杂志》2018年第1期31-34,共4页Journal of China Pediatric Blood and Cancer

基　　金：首都医科大学附属北京同仁医院院内基金-科研种子基金(编号:TRYY-KYJJ-2016-046)

摘　　要：目的分析三侧性视网膜母细胞瘤(TRB)的临床资料,探讨其诊断、治疗方法及预后情况。方法回顾性分析4例我院诊断为TRB的患儿临床特征及随访情况。结果4例TRB患儿中,1例有家族史(1/4);3例为双眼发病(3/4),l例为单眼发病(1/4);鞍旁占位1例,松果体占位2例,蝶鞍区占位1例;3例患儿首次血清神经元特异性烯醇化酶测得值升高,治疗后迅速降至正常。4例患儿中,2例经含有PECV方案化疗的规范治疗后病情均好转,其中1例颅内病灶消失,达完全缓解(CR),1例颅内病灶缩小,达部分缓解(PR);2例患儿因病情进展放弃治疗。截至随访日期,2例获随访患儿1例无事件生存,1例PR。结论TRB在临床上较为罕见,预后差,PECV方案化疗对其有效,但需更大样本进一步验证。Objective To analyze the clinical data of trilateral retinoblastoma, and investigate the diagnosis, treatment and prognosis. Methods Four cases of TRB patients diagnosed in our hospital were retrospectively reviewed their clinical presentation, treatment, and outcome were described. Results Among the 4 cases of TRB, only 1 patient was familial. Binocular were involved in 3 patients, and uniocular in 1 patient. The para saddle occupied 1 case, the pineal body occupied 2 cases, and the sella region occupied 1 case. NSE were elevated in 3 patients and returned to normal after treatment. Two patients was treated standardizedly, including PECV chemotherapy protocol, and improved gradually, one patient obtained complete response（ CR）, and 1 patient obtained partial response（ PR）. The other 2 patients abandoned treatment. In the follow-up, of the 2 patients who survived with tumors, one obtained partial response, the other 1 obtained progressive disease. Conclusions Trilateral retinoblastoma is rare and prognosis is poor. PECV chemotherapy protocol was effective, but still needs to be further validated by increasing sample numbers.

关 键 词：视网膜母细胞瘤 三侧性 双眼 肿瘤 

分 类 号：R739.7[医药卫生—肿瘤]