CT显示瘤体内无钙化的儿童视网膜母细胞瘤20例临床分析

Clinical analysis of 20 cases of retinoblastoma without calcification on CT scan

摘要 目的探讨CT显示瘤体内无钙化的视网膜母细胞瘤（RB）患儿的临床特点、治疗及预后。 方法选取2010年4月至2015年4月首都医科大学附属北京同仁医院诊治的710例RB患儿中，初次就诊CT提示瘤体内无钙化的20例患儿为研究对象。诊断方法：眼球摘除患儿根据病理结果进行确诊；未行眼球摘除的患儿根据临床表现、Retcam机眼底检查结果、眼B超等辅助检查，并结合化疗后复查眼底和影像学检查出现明确肿瘤缩小进行诊断。对其病例资料进行统计分析，总结其临床特点、治疗及预后情况；通过电话及门诊复查进行随访。 结果20例瘤体内无钙化的RB患儿中，男14例（70.0%），女6例（30.0%）；发病年龄〈5岁者19例（95.0%），〈3岁者14例（70.0%），〈1岁者1例（5.0%）；单眼发病者18例（90.0%），双眼发病2例（10.0%）；RB国际分期：D期17例（77.3%），E期4例（18.2%），C期1例（4.5%）。5例（25.0%）患儿直接手术摘除眼球，术后病理证实为RB；9例（45.0%）患儿行术前化疗，并定期复查眼底，发现玻璃体及网膜下瘤灶未消失或复发后，家长要求行眼球摘除术，术后病理证实为RB；5例（25.0%）化疗后肿瘤稳定，未行手术、定期复查眼底，1例（5.0%）患儿不规律化疗后未定期复查。14例行眼球摘除手术的患儿中，3例（21.4%）术后存在病理学高危因素，11例（78.6%）无病理学高危因素；未行眼球摘除的患儿，化疗后复查眼底均提示肿瘤缩小或出现钙化，12例复查眼眶CT出现钙化，临床上支持诊断为RB。随访结果：1例（5.0%）患儿失访，19例成功随访的患儿，定期复查均提示病情稳定。13例（68.4%）无病生存，6例（31.6%）带瘤生存。 结论对于眼眶CT显示瘤体内无钙化的疑似RB，应积极根据临床表现、Retcam机眼底检查结果、眼B超等辅助检查进行综合分析，及时诊断和治疗，减少误诊率，避免延误治疗。

Objective To analyze the clinicopathologic characteristics,treatment and prognosis of retinoblasto- ma without calcification. Methods The clinic and pathologic data of 20 cases of retinoblastoma without calcification were analyzed retrospectively. All the cases were selected from 710 cases of retinoblastoma archived in Beijing Tongren Hospital, Capital Medical University from April 2010 to April 2015. The inclusion criteria was no calcification on CT scan before treatment. The patients after enuclea-tion were diagnosed according to pathology; the patients without enu- cleation were diagnosed according to clinical manifestations, Reteam fundus, ophthalmic ultrasound and the reduction of tumor after chemotherapy. The clinical features, treatment and prognosis were summarized through statistical analysis of the 20 cases. The patients were followed - up through clinical reexamination and telephone calls. Results In this study,there were 14（70.0% ） male and 6（30.0% ） female patient＇s;there were 19 cases（95.0% ） less than 5 - year - old, 14 cases （70.0%） less than 3 - year - old, 1 case （5.0%） less than 1 - year - old. Eighteen cases （90.0%） were unilateral disease, and 2 cases （10.0%） were bilateral disease. According to international Intraocular Retinoblastoma Classification stage：4 cases （ 18.2% ） in stage E ,17 cases （77.3%） in stage D ,1 case （4.5%） in stage C. Five cases （ 25.0% ） were enucleated eyes, and pathology report was retinoblastoma. Nine cases （ 45.0% ） were treated with pre -enueleation chemotherapy, and finally enueleated after poor tumor shrinkage or recurrence, and the pathology was retinoblastoma. Five cases （25.0%） without enucleation were stable. One case （5.0%） failed to follow - up owing to the patients irregular treatment. In 14 enucleation cases, histopathologic high risk factors could be found in 3 cases （21.4%） ,and couldn＇t be found in the other 11 cases （78.6%）. In the cases that were not enuclea- ted ,the tumor diminished in size or new calcification presented in the fundus examination, and in 12 cases, new calcifi- cation was pre-sented on follow - up CT scan. Follow - up failed in 1 case（5.0% ） ,but follow - up was available for 19 cases,and among them 13 cases （68.4%） were disease -free survival and 6 cases （31.6%） were alive with tumor. Conclusions Close attention should be paid to the untypical retinoblastoma with no calcification on CT scan through well - taken medical history and meticulous imaging examination,in order to give early diagnosis and therapy.