视网膜母细胞瘤自发退行的临床组织病理学分析

Retinoblastoma spontaneous regression： clinical and histopathologic analysis

目的 分析视网膜母细胞瘤（RB）自发退行的临床表现和组织病理学特点.方法 回顾性系列病例研究.对北京同仁眼科中心病理室2007年1月至2013年12月间存档的579例因RB（术前未化疗）行眼球摘除的眼球标本中,表现为RB自发退行的52例的临床及组织病理学资料进行总结分析,主要分析了肿瘤细胞存活状况、肿瘤侵犯范围、葡萄膜和晶状体等眼内组织结构变化等情况.结果 52例RB患儿平均年龄为（14.0±8.7）个月,男性33例（63.5％）,女性19例（36.5％）.自发退行率为9.0％（52/579）,其中完全自发退行率为1.4％（8/579）.最常见的首发症状是白瞳征伴眼红29例（55.8％）,多数最终表现为眼球萎缩或眼球痨.典型的组织病理学特征：（1）眼球内肿瘤细胞全部或绝大多数发生凝固性坏死,仅见残存的肿瘤细胞残骸.（2）多数眼球体积明显萎缩变小或为眼球痨改变.（3）葡萄膜高度萎缩,色素细胞崩解,组织结构紊乱,尤其以虹膜、睫状体最为显著.（4）白内障,晶状体膨胀、移位、纤维结缔组织膜包裹.（5）23.1％ （12/52）的病例虽然眼球内的肿瘤组织已表现为典型的自发退行改变,然而,球后视神经内或脉络膜内仍可见有存活的肿瘤细胞,具有高危组织病理学因素,具有肿瘤向远处扩散、转移的风险.结论 应重视婴幼儿出现白瞳征伴眼红、眼球萎缩等不典型的临床表现,警惕RB自发退行的可能性;同时,对少数具有高危组织病理学因素的自发退行病例,术后应给予化学治疗等全身辅助治疗并密切随访观察.

Objective To analyze the clinicopathologic characteristics of retinoblastoma spontaneous regression.Methods Retrospective case series study.The clinic and pathologic data of 52 cases of retinoblastoma spontaneous regression were analyzed retrospectively.All the cases came from 579 cases of retinoblastoma （without pre-enucleation chemotherapy） archived in Ophthalmic Pathology Laboratory of Beijing Tongren Eye Centre from Jan.2007 to Dec.2013.The analyzed factors included tumor activity,tumor invasion,and tissue changes of uveal and lens.Results In this study,the patients＆#39; average age was （14.0 ± 8.7） months.There were 33 （63.5%） male and 19 （36.5%） female patients.The rate of retinoblastoma spontaneous regression was 9.0% （52/579）,of which the rate of complete regression was 1.4% （8/579）.The most common symptom was leucocoria coupled with red eye 55.8% （29/52）.The typical clinicopathologic characteristics were as follows：（1） all or most of all tumor cells suffered coagulation necrosis and only tumor cell remains were left; （2）most eyeballs showed atrophiabulbi or phthisis bulbi; （3） severe atrophy occurred in uvea,especially in iris and ciliary body combined with pigment cells disintegrating; （4） cataract formed coupled with lens swelling,displacement,or wrapped by fibrous connective tissue; （5） in a small number of cases,there were alive tumor cells in the retrolaminar optic nerve or choroid in spite of most tumor cells had suffered regression,indicating the patients had histopathologic high risk factors and had the dispositions of tumor diffusion and metastasis.Conclusions Some untypical clinical manifestation such as leukocoria coupled with red eye or atrophia bulbi should be paid close attention.The possibility of retinoblastoma spontaneous regression should be considered.Meanwhile,some cases of retinoblastoma spontaneous regression with histopathologic high risk factors should be given post-enucleation systemic adjuvant chemotherapy combined with close observations and follow-ups.