Acquaviva A, Ciccolallo L, Rondelli R, Balistreri A, Ancarola R, Cozza R, Hadjistilianou D, Francesco S De, Toti P, Pastore G, Haupt R, Carli M, Santoro N, Di Cataldo A, Fiorillo A, Indolfi P, Nucci P, Sandri A, Porta F, Porcaro A B, Tamaro P, Morgese G
Italian Retinoblastoma Registry, Department of Pediatrics, Obstetrics and Reproductive Medicine, University of Siena, Siena, Italy.
Oncogene. 2006 Aug 28;25(38):5350-7. doi: 10.1038/sj.onc.1209786.
Survivors of retinoblastoma (Rb) are at high risk of dying from second malignant tumour. The occurrence of second malignant neoplasm (SMN) and related mortality in a cohort of 1111 cases from the Italian Retinoblastoma Registry was analysed, considering the possible role of both genetic and iatrogenic causes. Rb patients had a greater than 10-fold excess in overall mortality compared with the general population (standardized mortality ratio (SMR) 10.73, 95% CI 9.00-12.80). Their excess risk attributable to cancers other than Rb was 14.93 95% CI 10.38-21.49). Survivors of hereditary Rb had an SMR for all causes of 16.25 (95% CI 13.20-20.00), whereas their SMR for all cancers was 25.72 (95% CI 17.38-38.07). Survivors of unilateral sporadic Rb had an SMR of 4.12 from all cancers (95% CI 1.55-10.98) and a much higher excess for overall mortality (SMR 13.34, 95% CI 10.74-16.56). As expected, survivors of hereditary Rb had higher mortality from cancers of the bone (SMR 391.90, 95% CI 203.90-753.20) and soft tissue (SMR 453.00, 95% CI 203.50-1008.40), small intestine (SMR 1375.50, 95% CI 344.00-5499.70), nasal cavity (SMR 13.71, 95% CI 1.93-97.35) and cancers of the brain and central nervous system (SMR 41.14, 95% CI 13.2-127.55).
视网膜母细胞瘤（Rb）幸存者死于第二原发性恶性肿瘤的风险很高。考虑到遗传和医源性原因的可能作用，对意大利视网膜母细胞瘤登记处的1111例病例队列中的第二原发性恶性肿瘤（SMN）的发生情况及相关死亡率进行了分析。与普通人群相比，Rb患者的总死亡率高出10倍以上（标准化死亡率（SMR）为10.73，95%置信区间为9.00 - 12.80）。其归因于Rb以外癌症的额外风险为14.93（95%置信区间为10.38 - 21.49）。遗传性Rb幸存者的所有原因的SMR为16.25（95%置信区间为13.20 - 20.00），而其所有癌症的SMR为25.72（95%置信区间为17.38 - 38.07）。单侧散发性Rb幸存者所有癌症的SMR为4.12（95%置信区间为1.55 - 10.98），总死亡率的额外风险则高得多（SMR为13.34，95%置信区间为10.74 - 16.56）。不出所料，遗传性Rb幸存者因骨癌（SMR为391.90，95%置信区间为203.90 - 753.20）、软组织癌（SMR为453.00，95%置信区间为203.50 - 1008.40）、小肠癌（SMR为1375.50，95%置信区间为344.00 - 5499.70）、鼻腔癌（SMR为13.71，95%置信区间为1.93 - 97.35）以及脑和中枢神经系统癌（SMR为41.14，95%置信区间为13.2 - 127.55）导致的死亡率更高。
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