视网膜母细胞瘤眼球摘除术后病理与预后分析

Analysis of histopathology and prognosis of retinoblastoma after enucleation

目的分析视网膜母细胞瘤（retinoblastoma，Rb）侵犯眼球部位与患者预后之间的关系，为病情的预测和治疗提供依据。方法回顾性分析2003年1月至2011年2月于中山大学中山眼科中心行眼球摘除术的单眼Rb患者。记录术后肿瘤侵犯的部位、治疗情况和生存情况并评估患者的5a生存率（5year probability event-free survival，5-PEFS）。随访以患者死亡或至2012年2月截止，随访时间为（46．3±29．2）个月。结果共有202例202眼患者纳入研究，其中筛板及筛板前侵犯所占比例最大（40．6％），5-PEFS也最高（96．1％）；筛板后视神经侵犯和视神经断端侵犯者5-PEFS分别是82．5％和40．0％，三者比较差异有显著统计学意义（P〈0．01）。筛板后视神经侵犯接受治疗的患者5-PEFS（91．6％）有高于未治疗者（66．7％）的趋势（P=0．09）；视神经断端侵犯者接受治疗与未治疗的5-PEFS分别是50．0％和0（P〈0．01），单纯脉络膜侵犯患者5-PEFS是93．8％，巩膜侵犯患者接受和未接受辅助化疗的5-PEFS分别是57．1％和77．8％（P〉0．05）。2例眼眶侵犯患者均死亡，4例患者眼球摘除术后眼眶复发，从眼球摘除术到眼眶复发的时间是5—15个月，平均为9．8个月。结论Rb眼球摘除术后病理检查对患者预后的预测和辅助治疗有指导意义；眼球摘除术后2a，尤其1a内是Rb复发的高发期，这期间所有患者需要密切随诊。

Objective To analyze the relationship between eyeball invasive locations and prognosis of retinoblastoma for metastasis after enucleation, and offer a reliable guide to adjuvant therapy. Methods Retrospective study was done about patients with unilateral retinoblastoma treated by enucleation without prior treatment at Zhongshan Ophthalmic Center of Sun Yat-Sen University between January 2003 and February 2011. The locations of tumor invasion,treatment and survival condition of patients were recorded, and the 5-PEFS （ 5 year probability event-free survival） was calculated. Follow-up visit ended until patients died or by February 2012. The follow-up period was （46.3± 29.2） months. Results A total of 202 eyes of 202 patients were enrolled in this study. The patients with the laminar or prelaminar involvement accounted for the largest proportion （40.6%） ,which had the highest 5-PEFS （96.1%）. The 5-PEFS of the patients with post-laminar optic nerve involvement and transection line of optic nerve involvement were 82.5% and 40.0% , respectively. The patients with post-laminar optic nerve involvement accepted adjuvant chemotherapy had a tendency of higher 5- PEFS （91.6%）than that of untreated patients （66.7% ,P =0.09）. The 5-PEFS of patients accepted treatment with transection line of optic nerve involvement significant higher than that of untreated patients（P 〈 0. 01 ）. The 5-PEFS of patients with isolated choroid invasion was 93.8%. The 5-PEFS of patients with tumor invasion to sclera who received adjuvant chemotherapy and that of those did not receive adjuvant chemothera- py were respectively 77.8% and 57. 1% （ P 〉 0.05 ）. Two patients with orbit invaded died. Four of 202 patients recurrented in orbit after enucleation. The average time from enucleation to relapse was 9.8 months （5 - 15 months）. Conclusion Histopathology after enucleation may provide a reference to prognosis and adjuvant therapy. Almost all recurrences of retinoblastoma occur within 2 years, especially 1 year, after enucleation, during which all retinoblastoma patients have to keeping close follow-up.