要评估呈现功能,肿瘤大小,以及晚期眼内视网膜母细胞瘤(RB)引起转移性死亡风险的治疗方法。
国际,多中心,基于注册表的回顾性病例系列。
总共1841例晚期RB患者。
高级RB由第8版美国癌症联合委员会(AJCC)类别cT2和cT3以及新的AJCC-眼科肿瘤学工作组(OOTF)规模组定义(1:<全球体积的50%,2:>50%,但<2/3,3:>2/3和4:扩散浸润RB)。治疗是初次摘除,全身化疗与二次摘除术,和眼部抢救的全身化疗。
转移性死亡。
cT2a患者级AJCC临床亚类的5年Kaplan-Meier累积生存率估计值为98%,cT2b为96%,88%为cT3a,95%为cT3b,cT3c为92%,84%的cT3d,和75%的cT3eRB。按治疗方式计算的生存率估计为96%,89%用于全身化疗和二次摘除术,和90%的全身化疗与眼部抢救。转移性死亡的风险随着cT亚类的增加而增加(P<0.001)。Cox比例风险回归分析证实,在cT3c类别中,转移性死亡率的风险更高(青光眼,危险比[HR],4.9;P=0.011),cT3d(眼内出血,HR,14.0;P<0.001),和cT3e(眼眶蜂窝织炎,HR,19.6;P<0.001)比cT2a类和具有继发性眼球摘除的全身化疗(HR,3.3;P<0.001)和眼部抢救(HR,4.9;P<0.001)比初次摘除。AJCC-OOTF大小组1至4的5年Kaplan-Meier累积生存率估计为99%,96%,94%,83%,分别。转移性RB的死亡率随着大小组的增加而增加(P<0.001)。Cox比例风险回归分析显示,第3组患者(HR,10.0;P=0.002)和4(HR,41.1;P<0.001)的转移性死亡风险高于第1组。
AJCC-RBcT2和cT3亚类和基于大小的AJCC-OOTF组3(>2/3眼球体积)和4(弥漫性浸润RB)提供了晚期眼内RB转移性死亡临床风险的可靠分层。原发性摘除术为晚期眼内RB患者提供了最高的生存率。
To evaluate presenting features, tumor size, and treatment methods for risk of metastatic death due to advanced intraocular retinoblastoma (RB).
International, multicenter, registry-based retrospective case series.
A total of 1841 patients with advanced RB.
Advanced RB was defined by 8th edition American Joint Committee on Cancer (AJCC) categories cT2 and cT3 and new AJCC-Ophthalmic Oncology Task Force (OOTF) Size Groups (1: < 50% of globe volume, 2: > 50% but < 2/3, 3: > 2/3, and 4: diffuse infiltrating RB). Treatments were primary enucleation, systemic chemotherapy with secondary enucleation, and systemic chemotherapy with eye salvage.
Metastatic death.
The 5-year Kaplan-Meier cumulative survival estimates by patient-level AJCC clinical subcategories were 98% for cT2a, 96% for cT2b, 88% for cT3a, 95% for cT3b, 92% for cT3c, 84% for cT3d, and 75% for cT3e RB. Survival estimates by treatment modality were 96% for primary enucleation, 89% for systemic chemotherapy and secondary enucleation, and 90% for systemic chemotherapy with eye salvage. Risk of metastatic mortality increased with increasing cT subcategory (P < 0.001). Cox proportional hazards regression analysis confirmed a higher risk of metastatic mortality in categories cT3c (glaucoma, hazard ratio [HR], 4.9; P = 0.011), cT3d (intraocular hemorrhage, HR, 14.0; P < 0.001), and cT3e (orbital cellulitis, HR, 19.6; P < 0.001) than in category cT2a and with systemic chemotherapy with secondary enucleation (HR, 3.3; P < 0.001) and eye salvage (HR, 4.9; P < 0.001) than with primary enucleation. The 5-year Kaplan-Meier cumulative survival estimates by AJCC-OOTF Size Groups 1 to 4 were 99%, 96%, 94%, and 83%, respectively. Mortality from metastatic RB increased with increasing Size Group (P < 0.001). Cox proportional hazards regression analysis revealed that patients with Size Group 3 (HR, 10.0; P = 0.002) and 4 (HR, 41.1; P < 0.001) had a greater risk of metastatic mortality than Size Group 1.
The AJCC-RB cT2 and cT3 subcategories and size-based AJCC-OOTF Groups 3 (> 2/3 globe volume) and 4 (diffuse infiltrating RB) provided a robust stratification of clinical risk for metastatic death in advanced intraocular RB. Primary enucleation offered the highest survival rates for patients with advanced intraocular RB.