Trilateral retinoblastoma:: A meta-analysis of hereditary retinoblastoma associated with primary ectopic intracranial retinoblastoma
Trilateral retinoblastoma:: A meta-analysis of hereditary retinoblastoma associated with primary ectopic intracranial retinoblastoma
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DOI：
10.1200/jco.1999.17.6.1829
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发表时间：
1999-06-01
影响因子：
45.3
通讯作者：
Kivelä, T
中科院分区：
文献类型：
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作者：
Kivelä, T
目的：为了获得关于三侧视网膜母细胞瘤（TRb）的精确知识，TRb是一种由遗传性视网膜母细胞瘤与颅内神经母细胞瘤相关的综合征，材料和方法：使用系统性文献综述，我们联系作者以获得缺失信息。对106例儿童视网膜母细胞瘤患者的资料进行Meta分析，包括频数分布和Kaplan-Meier生存曲线（范围：0至29个月）;与52名儿童（53%）的诊断年龄相比，47名儿童（47%）的诊断年龄更小散发性视网膜母细胞瘤（2 vs 6.5个月，P <0.0001）。TRb多累及第二代或第三代视网膜母细胞瘤。从视网膜母细胞瘤到TRb的中位时间为21个月（范围，术前6个月至术后141个月）; 78例（77%）松果体肿瘤的TRb时间长于23例（23%）鞍上肿瘤（32 vs 6.5个月，P <0.0001）。松果体和鞍上肿瘤的大小（27 v32 mm，P = .57）和预后（生存期9 v8个月，P = .91）相似。TRb检测较早（1对22个月，P = 0.0007），如果常规进行神经影像学检查，儿童存活时间较长（16对8个月，P = 0.001），但死亡年龄相似（36对37个月，P = 0.98）。如果进行筛查，累积5年生存率（可能表明治愈）为27%（v 0%）。所有儿童的TRB超过15毫米的大小dead.Conclusion：家族史，年龄诊断，和偏侧性视网膜母细胞瘤的儿童TRB类似：普通遗传性视网膜母细胞瘤。鞍上TRb比松果体TRb诊断更早，可能出现更早。当肿瘤直径小于1.5mm时，如能检出例TRb阳性者，则神经影像学筛查可提高治愈率。(C)1999年，美国临床肿瘤学会。
Purpose: To obtain refined knowledge regarding trilateral retinoblastoma (TRb), which is a syndrome that consists of hereditary retinoblastoma associated with an intracranial neuroblastic tumor,Materials and Methods: Using a systematic literature review, we contacted authors to obtain missing information. Data ham 106 children were used in a meta-analysis including frequency distributions and Kaplan-Meier survival curves.Results: TRb showed no sex predilection, Median age at diagnosis of retinoblastoma was 5 months (range, 0 to 29 months); age at diagnosis wets younger among 47 children (47%) with familiar retinoblastoma compared with age at diagnosis among 52 children (53%) with sporadic retinoblastoma (2 v 6.5 months, P < .0001). TRb usually affected the second or third generation with retinoblastoma. Median time from retinoblastoma to TRb was 21 months (range, 6 months before to 141 months after); time to TRb was longer for 78 (77%) pineal tumors compared with 23 (23%) suprasellar tumors (32 v 6.5 months, P < .0001). The size (27 v 32 mm, P = .57) and prognosis (survival of 9 v 8 months, P = .91) of pineal and suprasellar tumors were similar. TRb was detected earlier (1 v 22 months, P = .0007) and the child survived longer if neuroimaging was routinely performed (16 v 8 months, P = .001), but age at death was similar (36 v 37 months, P = .98). Cumulative 5-year survival (which was likely to indicate cure) was 27% (v 0%) if screening was undertaken. All children whose TRb exceeded 15 mm in size died.Conclusion: The family history, age at diagnosis,and laterality of retinoblastoma in children with TRb resembled that of: ordinary hereditary retinoblastoma. Suprasellar TRb were diagnosed earlier, and may arise earlier, than pineal TRb. Screening by neuroimaging could improve the cure rate if Eases of TRb were detected when tumors were 1 5 mm or smaller in size.(C) 1999 by American Society of Clinical Oncology.