Department of Radiation Oncology, Vanderbilt University School of Medicine, Nashville, Tennessee.
Pediatr Blood Cancer. 2014 Jan;61(1):116-9. doi: 10.1002/pbc.24714. Epub 2013 Aug 5.
As cure rates for retinoblastoma have improved, it is clear that patients with hereditary retinoblastoma experience increased risk of subsequent malignant neoplasms (SMNs).
Using the Surveillance, Epidemiology and End Results (SEER) database, we evaluated risk of SMNs in survivors or retinoblastoma. Standardized mortality ratios (SMRs) were calculated to compare number of deaths observed to the expected number for the cohort. Cumulative incidence of SMNs and standardized incidence ratios (SIRs) of observed to expected SMNs were calculated
A total of 595 patients were included in the analysis. Cumulative incidence of secondary malignancy at 30 years for patients with unilateral and bilateral disease was 1.7% and 28.5%, respectively (P < 0.001). SIRs of subsequent malignancies for patients with unilateral and bilateral disease were 2.1 (95% CI = 0.6-5.4) and 38.3 (95% CI = 24.3-57.5), respectively. Patients with bilateral disease treated with and without radiotherapy both experienced an increased risk of SMNs (SIRs = 45.9, 95% CI = 26.8-73.6 and 27.3, 95% CI = 10.0-59.4, respectively). The most common cause of death for the patients with bilateral disease was subsequent malignancy (52% of deaths). Beginning in the 1990s, there was a significant decrease in the use of radiotherapy as 30.5% of patients received radiotherapy in the 1980s compared to 2.6% after 1999 (P < 0.001).
Survivors of bilateral retinoblastoma experience an increased risk of SMNs which adversely affects survival. The use of radiotherapy in the management of retinoblastoma has declined; however, patients with bilateral disease remain at an increased risk of subsequent cancers.
随着视网膜母细胞瘤治愈率的提高，患有遗传性视网膜母细胞瘤的患者发生继发恶性肿瘤（SMN）的风险明显增加。
我们使用监测、流行病学和最终结果（SEER）数据库评估了视网膜母细胞瘤幸存者发生 SMN 的风险。通过计算标准化死亡率（SMR）来比较观察到的死亡人数与队列的预期死亡人数。计算继发恶性肿瘤的累积发生率和观察到的继发恶性肿瘤标准化发病率（SIR）。
共纳入 595 例患者进行分析。单侧和双侧疾病患者 30 年的继发恶性肿瘤累积发生率分别为 1.7%和 28.5%（P<0.001）。单侧和双侧疾病患者的继发恶性肿瘤 SIR 分别为 2.1（95%CI=0.6-5.4）和 38.3（95%CI=24.3-57.5）。接受和未接受放疗的双侧疾病患者均有发生 SMN 的风险增加（SIRs=45.9，95%CI=26.8-73.6 和 27.3，95%CI=10.0-59.4）。双侧疾病患者死亡的主要原因是继发恶性肿瘤（52%的死亡）。从 20 世纪 90 年代开始，放疗的应用显著减少，80 年代有 30.5%的患者接受放疗，而 1999 年后只有 2.6%（P<0.001）。
双侧视网膜母细胞瘤幸存者发生 SMN 的风险增加，这对生存产生不利影响。视网膜母细胞瘤治疗中放疗的应用减少；然而，双侧疾病患者仍有发生继发癌症的高风险。
