Yamanaka Ryuya, Hayano Azusa
Laboratory of Molecular Target Therapy for Cancer, Graduate School for Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Laboratory of Molecular Target Therapy for Cancer, Graduate School for Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.
World Neurosurg. 2017 May;101:722-730.e4. doi: 10.1016/j.wneu.2017.02.031. Epub 2017 Feb 16.
We conducted the largest systematic review of individual patient data to characterize secondary craniofacial sarcomas following retinoblastoma.
We conducted a systemic search of the PubMed databases and compiled a comprehensive literature review. Student t tests were used to evaluate differences between variables. Kaplan-Meier analysis was used to estimate survival. Statistical significance was assessed using a log-rank test.
We analyzed 220 cases of secondary craniofacial sarcomas, including 112 osteosarcomas. The average age (±SD) of onset for retinoblastoma was 1.20 ± 2.77 years. External-beam radiotherapy was delivered in 207 patients (94.0%) and chemotherapy was delivered in 53 patients (24.0%) patients. The latency period between retinoblastoma diagnosis and the onset of secondary sarcomas was 12 years. Cranial extension was found in 66 patients (30.0%). The median overall survival was worse with cranial extension (P = 0.0073). In cranial extended patients, the median survival in patients who received chemotherapy was 41 months, whereas patients who did not receive chemotherapy had a median survival of 12 months (P = 0.0020).
The risk of incidence of secondary sarcomas in retinoblastoma patients warrants longer follow-up periods. Moreover, chemotherapy should be considered as a potential treatment option for secondary cranial sarcomas following retinoblastoma.
我们开展了规模最大的个体患者数据系统评价，以描述视网膜母细胞瘤后继发性颅面部肉瘤的特征。
我们对PubMed数据库进行了系统检索，并汇编了一篇全面的文献综述。采用学生t检验评估变量之间的差异。采用Kaplan-Meier分析估计生存率。使用对数秩检验评估统计学显著性。
我们分析了220例继发性颅面部肉瘤病例，其中包括112例骨肉瘤。视网膜母细胞瘤的平均发病年龄（±标准差）为1.20±2.77岁。207例患者（94.0%）接受了外照射放疗，53例患者（24.0%）接受了化疗。视网膜母细胞瘤诊断与继发性肉瘤发病之间的潜伏期为12年。66例患者（30.0%）发现有颅骨扩展。颅骨扩展患者的总生存期中位数更差（P = 0.0073）。在颅骨扩展患者中，接受化疗的患者中位生存期为41个月，而未接受化疗的患者中位生存期为12个月（P = 0.0020）。
视网膜母细胞瘤患者发生继发性肉瘤的风险需要更长的随访期。此外，化疗应被视为视网膜母细胞瘤后继发性颅骨肉瘤的一种潜在治疗选择。
