Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA.
Ophthalmology. 2013 May;120(5):997-1003. doi: 10.1016/j.ophtha.2012.10.044. Epub 2013 Feb 8.
To determine the correlation between the International Classification of Retinoblastoma (ICRB) and histopathologic high-risk retinoblastoma.
Retrospective study.
A total of 519 patients.
Primary enucleation.
High-risk retinoblastoma, metastasis, and death.
Of 519 primarily enucleated eyes, 87 (17%) were classified as group D and 432 (83%) were classified as group E on the basis of the ICRB. High-risk retinoblastoma was identified in 23% (117/519) of enucleated eyes, including 17% (15/87) group D and 24% (102/432) group E eyes. High-risk histopathologic features of retinoblastoma included anterior chamber involvement (5/15 [33%] group D eyes, 31/102 [30%] group E eyes), isolated massive posterior uveal invasion ≥ 3 mm (7/15 [47%] group D eyes, 22/102 [22%] group E eyes), isolated post-laminar optic nerve invasion (2/15 [13%] group D eyes, 46/102 [45%] group E eyes), and any combination of posterior uveal invasion and optic nerve involvement (7/15 [47%] group D eyes, 37/102 [36%] group E eyes). On logistic regression analysis, massive posterior uveal invasion ≥ 3 mm was more common in group D eyes (P = 0.0442), and post-laminar optic nerve invasion was more common in group E eyes (P = 0.0390). Of 117 patients with high-risk retinoblastoma, systemic adjuvant chemotherapy was administered in 83 patients (71%). Systemic metastasis developed in 0% (0/15) of those with high-risk group D retinoblastoma and 10% (10/102) of those with high-risk group E retinoblastoma over a mean follow-up period of 78 months (median, 62 months; range, 1-419 months). There was no metastasis in any patient (n = 402) classified with non-high-risk retinoblastoma. Of the 10 patients who developed metastasis, 4 had received prior adjuvant chemotherapy and 6 had no prior adjuvant chemotherapy. There was no metastasis in high-risk patients treated with vincristine sulphate, etoposide phosphate, and carboplatin (VEC). Death from metastasis occurred in 4% of high-risk patients (5/117).
On the basis of the ICRB, 17% of group D and 24% of group E eyes are at increased risk for metastatic disease. In this study, 8% of patients developed metastasis. There was no metastasis in any patient classified with non-high-risk retinoblastoma.
FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
确定国际视网膜母细胞瘤分类（ICRB）与组织病理学高危视网膜母细胞瘤之间的相关性。
回顾性研究。
共计 519 例患者。
初次眼球摘除术。
高危视网膜母细胞瘤、转移和死亡。
在基于 ICRB 进行分类的 519 例初次眼球摘除的眼中，87 例（17%）为 D 组，432 例（83%）为 E 组。519 例眼球中，有 23%（117/519）被诊断为高危视网膜母细胞瘤，包括 17%（15/87）的 D 组和 24%（102/432）的 E 组。高危组织病理学特征包括前房受累（5/15 [33%] D 组眼，31/102 [30%] E 组眼）、孤立性后部葡萄膜侵袭≥3mm（7/15 [47%] D 组眼，22/102 [22%] E 组眼）、孤立性后层视神经侵袭（2/15 [13%] D 组眼，46/102 [45%] E 组眼）以及任何后部葡萄膜侵袭和视神经受累的组合（7/15 [47%] D 组眼，37/102 [36%] E 组眼）。多变量逻辑回归分析显示，D 组中后部葡萄膜侵袭≥3mm更为常见（P=0.0442），E 组中后层视神经侵袭更为常见（P=0.0390）。在 117 例高危视网膜母细胞瘤患者中，83 例（71%）接受了全身辅助化疗。在平均随访 78 个月（中位数 62 个月；范围 1-419 个月）期间，高危 D 组视网膜母细胞瘤患者中无一例发生全身转移（0/15），高危 E 组视网膜母细胞瘤患者中 10%（10/102）发生全身转移。任何非高危视网膜母细胞瘤患者（n=402）均未发生转移。在发生转移的 10 例患者中，4 例接受了辅助化疗，6 例未接受辅助化疗。接受硫酸长春新碱、依托泊苷磷酸酯和卡铂（VEC）治疗的高危患者无一例发生转移。4%（5/117）的高危患者死于转移。
基于 ICRB，17%的 D 组和 24%的 E 组眼有发生转移疾病的风险增加。在本研究中，8%的患者发生了转移。任何非高危视网膜母细胞瘤患者均未发生转移。
