Screening for retinoblastoma: presenting signs as prognosticators of patient and ocular survival
Screening for retinoblastoma: presenting signs as prognosticators of patient and ocular survival
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DOI：
10.1542/peds.112.6.1248
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发表时间：
2003-12-01
期刊：
影响因子：
8
通讯作者：
Ranjithan, M
中科院分区：
文献类型：
--
作者：
Abramson, DH;Beaverson, K;Ranjithan, M
Objective.将视网膜母细胞瘤的3种常见表现与患者和眼部存活率相关联，并评估目前儿童视网膜母细胞瘤筛查实践的有效性。回顾性研究了1831例视网膜母细胞瘤患者从我们的中心（1914年至2000年6月）。采用Kaplan-Meier方法计算和分析患者生存率（不包括其他原发性肿瘤导致的死亡）和眼部生存率（患眼）。白瞳与极好的患者生存率（> 86%，5年）相关，但单侧（4%，5年）和双侧患者（29%，5年）的眼部生存率较差。1654例患者中共有308例（19%）出现斜视：患者生存率极佳（90%，5年），眼部生存率较差（17%，5年），但优于白瞳。有视网膜母细胞瘤家族史并从出生时就进行视网膜肿瘤临床筛查的患者被诊断为更年轻（8个月大）和更早（Reese Ellsworth组1 = 45人中的26人[58%]），并且比有家族史的未筛查患者的眼部生存率更高。更多的患者最初是由家人/朋友（1315/1632 [ 80%]）发现的，而不是儿科医生（123/1632 [8%]）或眼科医生（156/1632 [10%]）。大多数被诊断为视网膜母细胞瘤的美国儿童最初表现为父母检测到的白瞳，尽管通过红色反射试验进行了常规的白瞳筛查。白瞳或斜视时的初始疾病检测与高患者存活率和存在眼的低眼存活率相关。挽救眼睛和视力需要在白瞳症之前进行疾病识别，如具有阳性家族史并通过眼科医生进行早期常规扩瞳眼底镜检查进行临床监测的患者的更好的眼睛挽救率所证明的那样。
Objective. To correlate 3 common presenting signs of retinoblastoma with patient and ocular survival and to assess the efficacy of current pediatric screening practices for retinoblastoma.Methods. A retrospective study was conducted of 1831 retinoblastoma patients from our center ( 1914 - June 2000). Patient survival ( excluding deaths from other primary neoplasms) and ocular survival ( presenting eyes) rates were calculated and analyzed using the Kaplan-Meier method.Results. Leukocoria correlated with excellent patient survival (> 86%, 5 years) but poor ocular survival in unilateral (4%, 5 years) and bilateral patients (29%, 5 years). A total of 308 (19%) of 1654 patients presented with strabismus: patient survival was excellent (90%, 5 years), and ocular survival was poor (17%, 5 years) yet better than leukocoria. Patients who had a family history of retinoblastoma and were clinically screened for retinal tumors from birth were diagnosed younger ( 8 months of age) and earlier (Reese Ellsworth group 1 = 26 [58%] of 45) and had better ocular survival than nonscreened patients with a family history. More patients were initially detected by family/ friends ( 1315 [ 80%] of 1632) than pediatricians ( 123 [8%] of 1632) or ophthalmologists ( 156 [10%] of 1632).Conclusion. Most US children whose retinoblastoma is diagnosed initially present with leukocoria detected by a parent, despite routine pediatric screening for leukocoria via the red reflex test. Initial disease detection at the point of leukocoria or strabismus correlated with high patient survival rates and poor ocular survival rates for the presenting eye. Saving eyes and vision requires disease recognition before leukocoria, as demonstrated by the better ocular salvage rate among patients who had a positive family history and received clinical surveillance via early, routine dilated funduscopic examinations by an ophthalmologist.