Demirci Hakan, Eagle Ralph C, Shields Carol L, Shields Jerry A
Oncology Service and the Department of Pathology, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA 19107, USA.
Arch Ophthalmol. 2003 Aug;121(8):1125-31. doi: 10.1001/archopht.121.8.1125.
To evaluate the histopathologic findings in the eyes with retinoblastoma that had been treated only with chemoreduction.
Clinicopathologic series. Study Material Ten eyes of 8 patients with retinoblastoma that were enucleated after therapy consisting only of systemic chemotherapy (chemoreduction).
All cases received a chemoreduction regimen including a combination of intravenous carboplatin, etoposide phosphate, and vincristine sulfate. Adjuvant treatment to the tumor was not provided in any case. The enucleated globes were studied by routine light microscopy. Main Outcome Measure Histopathologic features of retinoblastoma following chemoreduction.
At presentation, there were 8 eyes in Reese-Ellsworth group V, 1 eye in group IV, and 1 eye in group III. After chemoreduction (mean, 4 cycles; range, 1-6 cycles), the main tumor regressed a mean 34% in thickness and 24% in basal diameter. The indication for enucleation was retinoblastoma recurrence as subretinal and/or vitreous seeds in 7 eyes and extensive vitreous hemorrhage in 3 with uncertainty about viable-appearing tumor. In no case was enucleation performed for recurrence of the main tumor. In all eyes, there was histopathologic evidence of tumor regression. In 8 of 10 eyes, histopathologic examination disclosed tumor regression without viable-appearing retinoblastoma in the main tumor. Of these 8 eyes, 2 showed a completely calcified glial scar and 6 showed an apical calcified glial scar and a basal residual well-differentiated component with retinomalike and/or retinocytomalike features. In the remaining 2 eyes, an area of posttherapeutic regression was present but contained foci of mitotically active, viable-appearing malignant retinoblastoma cells. The 6 eyes found to contain well-differentiated component with retinomalike and/or retinocytomalike features showed a mean decrease of 17% in largest basal dimension and 32% in thickness after a mean of 3 cycles of chemoreduction. In contrast, the 4 eyes that did not contain well-differentiated component with retinomalike and/or retinocytomalike features showed a mean decrease of 35% in largest basal dimension and 55% in thickness after a mean of 5 cycles of chemoreduction. Of those 7 eyes enucleated for recurrent subretinal and/or vitreous seeds, viable tumor seeds were confirmed histopathologically in all cases. There was no histopathologic evidence of chemotherapeutic toxicity to the eye.
Histopathologic examination of 10 enucleated eyes following chemoreduction alone revealed that the main retinoblastoma regressed in all eyes. Additionally 6 eyes showed basal residual well-differentiated component with retinomalike and/or retinocytomalike features, and these eyes also displayed less shrinkage with chemoreduction. Despite the lack of viable-appearing retinoblastoma within the main tumor, enucleation was performed for viable subretinal and/or vitreous seeds in 7 cases and confirmed histopathologically.
评估仅接受化学减灭术治疗的视网膜母细胞瘤患眼的组织病理学表现。
临床病理系列研究。研究材料8例视网膜母细胞瘤患者的10只患眼，这些患眼在仅接受全身化疗（化学减灭术）后被摘除眼球。
所有病例均接受化学减灭术方案，包括静脉注射卡铂、磷酸依托泊苷和硫酸长春新碱联合应用。所有病例均未给予肿瘤辅助治疗。对摘除的眼球进行常规光学显微镜检查。主要观察指标化学减灭术后视网膜母细胞瘤的组织病理学特征。
初诊时，Reese-Ellsworth V组有8只眼，IV组有1只眼，III组有1只眼。化学减灭术后（平均4个周期；范围1 - 6个周期），主要肿瘤厚度平均消退34%，基底直径平均消退24%。摘除眼球的指征为视网膜下和/或玻璃体内种植的视网膜母细胞瘤复发7只眼，以及广泛玻璃体积血3只眼，且难以确定肿瘤是否存活。主肿瘤复发未行任何一例眼球摘除。所有患眼中均有肿瘤消退的组织病理学证据。10只眼中的8只，组织病理学检查显示主肿瘤消退，未见存活的视网膜母细胞瘤。在这8只眼中，2只显示完全钙化的胶质瘢痕，6只显示顶端钙化的胶质瘢痕和基底残留的分化良好成分，具有视网膜样和/或视网膜细胞瘤样特征。其余2只眼中，存在治疗后消退区域，但含有有丝分裂活跃、存活的恶性视网膜母细胞瘤细胞灶。发现6只眼含有具有视网膜样和/或视网膜细胞瘤样特征的分化良好成分，在平均3个周期的化学减灭术后，最大基底径平均减小17%，厚度平均减小32%。相比之下，4只眼未含有具有视网膜样和/或视网膜细胞瘤样特征的分化良好成分，在平均5个周期的化学减灭术后，最大基底径平均减小35%，厚度平均减小55%。在因视网膜下和/或玻璃体内种植复发而摘除的7只眼中，所有病例经组织病理学证实存在存活的肿瘤种植。没有化学治疗对眼毒性的组织病理学证据。
对10只仅接受化学减灭术后摘除的眼球进行组织病理学检查发现，所有患眼的主要视网膜母细胞瘤均消退。另外6只眼显示基底残留的分化良好成分，具有视网膜样和/或视网膜细胞瘤样特征，这些眼在化学减灭术后缩小程度也较小。尽管主肿瘤内未见存活的视网膜母细胞瘤，但7例因视网膜下和/或玻璃体内有存活种植灶而进行了眼球摘除，并经组织病理学证实。
