Department of Ophthalmology, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Department of Pathology, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Int Ophthalmol. 2020 Nov;40(11):2875-2887. doi: 10.1007/s10792-020-01472-8. Epub 2020 Jul 6.
The management of bilateral advanced retinoblastoma (RB) cases is challenging with attempts to use neoadjuvant therapy salvaging of one of the globes. Our aim in this study was to demonstrate the effect of this primary therapy on the histopathological features and risk factors in secondary enucleated compared to primarily enucleated globes with groups D and E RB.
We retrospectively reviewed all enucleated globes with advanced RB received in the pathology laboratories over a period of 5 years. Patients were divided into two groups: one with primary enucleations and another with at least one secondary enucleated globe, and their demographic and clinical data were analyzed. The enucleated globes in the two groups were analyzed to compare the general histopathological features including tumor seeding, size, differentiation, growth pattern, mitotic figures, and focality. More importantly, high-risk features: choroidal invasion, optic nerve (ON) invasion, iris/anterior chamber invasion, ciliary body invasion, and scleral and extra-scleral extension, as well as the pathological classification of the tumor (pT) according to the American Joint Committee on Cancer 7th edition were also compared between the two groups.
We had a total of 106 enucleated globes (78 primary and 28 secondary enucleations) from 99 patients with advanced RB (73 patients with primarily and 26 with secondarily enucleated globes). Demographic and clinical profiles of patients were similar in both, but the mean interval from presentation to enucleation was significantly longer in the secondary enucleations (P = 0.015). Rare/occasional mitotic figures were observed in secondary enucleations using multivariate analysis (P = 0.003). Primarily enucleated globes had higher risk of tumor seeding (P = 0.020), post-laminar/surgical margin ON invasion (P = 0.001), and massive choroidal invasion (P = 0.028). Half of the secondary enucleated globes had tumors confined to the globes without invasion (pT1) and statistically significant lower tumor classifications (pT1 or pT2a) compared to primary enucleations (P =0.001). However, 18% of the secondarily enucleated globes in 3 patients had unfavorable outcome with RB-related mortality after a period of 1-4 years.
Secondary enucleated globes with advanced RB show favorable histopathological findings mainly less mitosis. These eyes have significantly lower chance for harboring choroidal and ON invasion, thus mostly classified as pT1 or pT2a when compared to primarily enucleated globes. The decision for secondary enucleation was observed to be significantly delayed (8.0 months ± 9.8). Prompt decision for needed enucleation based on the response to primary treatment and careful histopathological examination of enucleated globes are essential to prevent disease-related mortality.
对于双侧晚期视网膜母细胞瘤（RB）病例的管理，尝试使用新辅助治疗来挽救一只眼球具有挑战性。我们在这项研究中的目的是展示这种主要治疗方法对组织病理学特征的影响，并对 D 组和 E 组 RB 患者的继发眼球与原发性眼球进行比较。
我们回顾性分析了在过去 5 年中在病理实验室接收的所有接受过晚期 RB 治疗的眼球。患者分为两组：一组是初次眼球摘除组，另一组是至少有一次二次眼球摘除的组，并分析了他们的人口统计学和临床数据。比较两组的一般组织病理学特征，包括肿瘤播种、大小、分化、生长模式、有丝分裂计数和局灶性。更重要的是，还比较了两组之间具有高风险特征：脉络膜侵犯、视神经（ON）侵犯、虹膜/前房侵犯、睫状体侵犯、巩膜和眼外侵犯，以及根据美国癌症联合委员会第 7 版的肿瘤病理分类（pT）。
我们共对 99 例晚期 RB 患者（73 例初次眼球摘除，26 例二次眼球摘除）的 106 个眼球进行了眼球摘除（78 个初次眼球摘除，28 个二次眼球摘除）。两组患者的人口统计学和临床特征相似，但二次眼球摘除的平均就诊至眼球摘除间隔时间明显更长（P=0.015）。多变量分析显示，二次眼球摘除中偶尔出现罕见的有丝分裂计数（P=0.003）。初次眼球摘除的眼球更有可能发生肿瘤播种（P=0.020）、后层/手术边缘 ON 侵犯（P=0.001）和大量脉络膜侵犯（P=0.028）。一半的二次眼球摘除的眼球肿瘤局限于眼球内，无侵犯（pT1），且与初次眼球摘除相比，肿瘤分类明显较低（pT1 或 pT2a）（P=0.001）。然而，在 3 名患者中，18%的二次眼球摘除的眼球在 1-4 年后因与 RB 相关的死亡而出现不良预后。
晚期 RB 的二次眼球摘除显示出有利的组织病理学发现，主要是有丝分裂计数较少。这些眼睛发生脉络膜和 ON 侵犯的可能性显著降低，因此与初次眼球摘除相比，大多被归类为 pT1 或 pT2a。二次眼球摘除的决定明显延迟（8.0 个月±9.8）。基于对初次治疗的反应和对眼球摘除物的仔细组织病理学检查，及时决定是否需要眼球摘除，对于预防与疾病相关的死亡至关重要。
