Department of Radiation Oncology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1 of Shuaifuyuan Road, Dongcheng District, Beijing, 100730, China.
Radiat Oncol. 2021 Nov 27;16(1):228. doi: 10.1186/s13014-021-01943-x.
Neuroblastoma is a common extracranial malignant tumor in children. Its main treatment modality is a combination of chemotherapy, radiotherapy, and surgery. Given the advances in chemotherapy regimens and the widespread use of bone marrow transplantation over the decades, there has been improvement in treatment efficacy, which has led to prolonged patient survival. Accordingly, long-term complications have become a growing concern among physicians and patients. This study aimed to analyze the survival rate of patients with neuroblastoma and the risk factors for developing second malignant neoplasms (SMNs).
The SEER 18 Regs (1973-2015) and SEER 9 Regs (1973-2015) data of the surveillance, epidemiology, and end results (SEER) database of the US National Cancer Institute were adopted for survival and SMN analysis.
The 5-, 10-, and 20-year overall survival rates of patients with neuroblastoma were 67%, 65%, and 62%, respectively. Among 38 patients with neuroblastoma who presented with SMNs, those with abdomen as the primary site accounted for the majority (63.2%), followed by those with thorax (26.3%) and other sites (10.5%). SMNs occurred more commonly in non-specific neuroblastoma (incidence: 0.87%) than ganglioneuroblastoma (incidence: 0.3%). Compared with the general population, the risk of SMN is significantly higher (SIR = 4.36). The risk of developing SMNs was significantly higher in the digestive system (SIR = 7.29), bones and joints (SIR = 12.91), urinary system (SIR = 23.48), brain and other nervous systems (SIR = 5.70), and endocrine system (SIR = 5.84). Multivariate analysis revealed that the year of diagnosis (OR = 2.138, 95% CI = 1.634-2.797, p < 0.001) was the only independent risk factor for developing SMNs.
This study identifies the risk factor for developing SMNs in patients with neuroblastoma, which could facilitate individualized screening for high-risk patients, to allow early diagnosis and treatment of SMNs.
神经母细胞瘤是儿童常见的颅外恶性肿瘤。其主要治疗方式为化疗、放疗和手术相结合。几十年来，随着化疗方案的进步和骨髓移植的广泛应用，治疗效果得到了改善，这导致患者的生存时间延长。因此，长期并发症成为医生和患者越来越关注的问题。本研究旨在分析神经母细胞瘤患者的生存率和发生第二恶性肿瘤（SMN）的风险因素。
本研究采用美国国家癌症研究所监测、流行病学和最终结果（SEER）数据库的 SEER 18 规则（1973-2015 年）和 SEER 9 规则（1973-2015 年）数据进行生存和 SMN 分析。
神经母细胞瘤患者的 5 年、10 年和 20 年总生存率分别为 67%、65%和 62%。在 38 例发生 SMN 的神经母细胞瘤患者中，原发部位为腹部的占多数（63.2%），其次为胸部（26.3%）和其他部位（10.5%）。SMN 更常见于非特异性神经母细胞瘤（发病率：0.87%），而神经节母细胞瘤（发病率：0.3%）则少见。与普通人群相比，SMN 的发病风险明显更高（SIR=4.36）。消化系统（SIR=7.29）、骨骼和关节（SIR=12.91）、泌尿系统（SIR=23.48）、脑及其他神经系统（SIR=5.70）和内分泌系统（SIR=5.84）的 SMN 发病风险明显更高。多变量分析显示，诊断年份（OR=2.138，95%CI=1.634-2.797，p<0.001）是发生 SMN 的唯一独立风险因素。
本研究确定了神经母细胞瘤患者发生 SMN 的风险因素，这有助于对高危患者进行个体化筛查，以便早期诊断和治疗 SMN。
