目的 探讨视网膜母细胞瘤(RB)化学减容加局部治疗后的临床消退模式及预后.方法 回顾性系列病例研究.分析2005年1月至2009年6月间于复旦大学附属眼耳鼻喉科医院接受化学减容加局部治疗的RB患儿37例47只眼,122个RB瘤体.其中男性27例,女性10例.平均年龄为22个月.化学减容加局部治疗以长春新碱、依托泊甙、卡铂联合应用方案化学减容,辅以冷冻、经瞳孔温热疗法等局部巩固治疗.随访时间为12～60个月,平均32个月.视网膜母细胞瘤的消退模式包括0型:完全消退无痕迹；1型:完全钙化；2型:无钙化；3型:部分钙化；4型:萎缩瘢痕.对于存在家族史与否的患儿每只眼瘤体数的统计学差异采用秩和检验,对于不同肿瘤厚度及肿瘤位置肿瘤消退模式的差异采用x2检验.采用多因素回归分析各型肿瘤消退模式和患者发病年龄、患者性别、肿瘤大小、肿瘤部位、是否存在家族史的关系.结果 47只眼参照眼内型视网膜母细胞瘤国际分期分为A期20只眼(42.6％),B期13只眼(27.6％),C期6只眼(12.8％),D期8只眼(17.0％).122个瘤体平均每只眼瘤体数为2.6个.消退模式为0型3个,1型15个,2型8个,3型25个,4型71个.肿瘤厚度和肿瘤位置与肿瘤消退模式相关(x2 =86.52,21.15;P=0.000,0.007),初始肿瘤厚度＜2 mm的瘤体常见4型消退模式,厚度＞8 mm瘤体常见1型和3型消退模式.远离黄斑区的瘤体多见4型消退模式.多因素同归分析显示1型消退模式的预测因素为肿瘤厚度＞8 mm(Z =3.02·P =0.003).3型消退模式的预测因素为发病年龄大、肿瘤厚度＞8 mm和非赤道部-锯齿缘肿瘤(Z=3.98,2.23,3.60;P=0.000,0.025,0.000).4型消退模式的预测因素为存在家族史的患儿、肿瘤厚度＜2 mm及赤道部,锯齿缘的肿瘤(Z =4.37,3.42,2.42；P =0.000,0.000,0.021).12个瘤体复发,其中9个为3型消退模式,3个为4型消退模式.8只眼产生15个新瘤体.5例发生新肿瘤的患者均为发病年龄小和有家族史的患者.复发和产生新瘤体的时间平均为化疗结束后6个月.结论 化学减容加局部治疗后3型和4型消退模式最常见.小肿瘤常见4型消退模式,较大瘤体常见l型和3型消退模式.3型和4型消退模式肿瘤可能易复发.发病年龄小和有家族史的患者较易发生新肿瘤.接受化学减容加局部治疗的患者应进行密切随访.%Objective To evaluate retinoblastoma regression patterns following chemoreduction and adjuvant therapy.Methods Retrospective case series.122 tumors of 47 eyes of 37 patients following chemoreduction and adjuvant therapy between January 2005 and June 2009 in the Eye & ENT hospital of Fudan University.Twenty-seven patients are male,and 10 Patients are female. The average age was 22 months.The combined therapy included chemoreduction using vincristine,etoposide,and carboplatin ( VEC ) combined with local cryotherapy and/or transpupillary thermotherapy (TTT).The average follow-up duration was 32 months ranging from 12 to 60 months.Regression patterns included type 0 ( no remnant),type 1 (calcified remnant),type 2 (noncalcified remnant),type 3 (partially calcified remnant),and type 4 ( flat scar).Wilcoxon rank sum test was used to test the difference of tumor number between the patients with family history and those without family history.Chi-square test was used to test the difference between the tumor thickness,tumor location and regression patterns.Multivariate logistic regression analysis was used to test the correlation between the regression patterns and age,sex,tumor thickness,tumor location and family history.Statistical significance was assigned at P ＜ 0.05.Results Forty-seven eyes according to the International Intraocular Retinoblastoma Classification,20 eyes (42.6％ ) were group A,13 eyes (27.6％)group B,6 eyes ( 12.8％ ) group C,8 eyes ( 17.0％ ) group D.Of 122 tumors,the average number of tumors per eye was 2.6.Retinoblastoma regressions were type 0 ( n =3 ),type 1 ( n-15 ),type 2 ( n =8),type 3 (n =25),and type 4 (n =71 ).Tumor thickness and tumor location were related to regression patterns.Tumors with an initial thickness of 2 mm or less regressed most often to type 4,and those thicker than 8 mm regressed to type 1 or type 3.Tumors with greater distance from the foveola regressed most often to type 4.The factors predictive of regression pattern type 1 included tumor thickness larger than 8 mm ( Z =3.02,P =0.003 ).The factors predictive of regression pattern tvpe 3 included older age,tumor thickness larger than 8 mm and location not in the equator to ors serrata region( Z =3.98,2.23,3.60;P =0.000,0.025,0.000 ).The factors predictive of regression pattern type 4 included familial hereditary pattern,tumor thickness smaller than 2 mm and location in the equator to ors serrata region.(Z =4.37,3.42,2.42 ; P =0.000,0.000,0.021 ).12 tumors recurred,9 tumors were type 3 and 3 tumors were type 4.8 eyes developed 15 new tumors.5 patients developed new tumors were all younger patients and had familial hereditary history.The average period of recurrence of main tumors and development of new tumors was six months after the end of chemoreduction. Conclusiots Following chemoreduction,type 3 and type 4 regression patterns were most common.Smaller tumors were usually seen in type 4,and bigger tumors were usually seen in type 1 or type 3.Tumor recurrence was usually found following regression pattern type 3 or type 4.Younger patients and patients with familial hereditary history trended to develop new tumors.Patients accept chemoreduction and adjuvant therapy need close follow-up.
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