Osteosarcoma following retinoblastoma: age at onset and latency period.
Osteosarcoma following retinoblastoma: age at onset and latency period.
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DOI：
10.1076/opge.22.2.77.2228
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发表时间：
2001-06-01
影响因子：
1.2
通讯作者：
Dutrillaux, B
中科院分区：
文献类型：
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作者：
Chauveinc, L;Mosseri, V;Dutrillaux, B
为了评估遗传易感性在诱导放射性肿瘤中的作用，我们对文献和我们自己研究所的数据进行了统计分析，分析了骨肉瘤作为视网膜母细胞瘤第二原发肿瘤的发病年龄和潜伏期，无论是否进行后续放射治疗。在视网膜母细胞瘤幸存者谁后来发展成骨肉瘤，在视网膜母细胞瘤的发病年龄是年轻的（平均12个月），在单侧和双侧的形式。这表明，所有或几乎所有的患者都是由RB1基因的一个等位基因突变的遗传易感性。对于视网膜母细胞瘤患者，骨肉瘤发生在辐射野内比辐射野外早1.2年。放疗与骨肉瘤发病的潜伏期在照射野内比照射野外短1.3年。有趣的是，双峰分布的潜伏期内出现的骨肉瘤观察到，但不是外的辐射场：40%的发生后，一个短的潜伏期，而其余60%的潜伏期与骨肉瘤发生外的辐射场。这表明不同的机制可能参与放射致癌作用。辐射诱导的第二个RB1等位基因突变可能是骨肉瘤在短时间延迟后发生的原因，而其他基因可能会在较长时间延迟后发生。
In order to assess the role of genetic predisposition in the induction of radiation-induced tumors, we performed statistical analysis on data from the literature and from our own Institute with regard to the age at onset and the latency period of osteosarcoma as the second primary tumor for retinoblastoma with or without subsequent radiotherapy. In retinoblastoma survivors who subsequently developed osteosarcoma, the age at onset of retinoblastoma was young (average of 12 months) in both unilateral and bilateral forms. This suggests that all or almost all of the patients were genetically predisposed by a mutation of one allele of the RB1 gene. For retinoblastoma patients, osteosarcomas occurred 1.2 years earlier inside than outside the radiation field. The latency period between radiotherapy and osteosarcoma onset was 1.3 years shorter inside than outside the radiation field. Interestingly, a bimodal distribution of latency periods was observed for osteosarcomas arising inside, but not outside the radiation field: 40% occurred after a short latency, while the latency of the remaining 60% was comparable to that of osteosarcoma occurring outside the radiation field. This suggests that different mechanisms may be involved in radiocarcinogenesis. A radiation-induced mutation of the second RB1 allele may be the cause of osteosarcomas occurring after a short delay, while other genes may be affected in those occurring after a longer delay.