Gutierrez Juan C, Fischer Anne C, Sola Juan E, Perez Eduardo A, Koniaris Leonidas G
Division of Surgical Oncology, DeWitt Daughtry Family Department of Surgery, University of Miami Miller School of Medicine, Miami, FL 33136, USA.
Pediatr Surg Int. 2007 Jul;23(7):637-46. doi: 10.1007/s00383-007-1933-7. Epub 2007 May 3.
We sought to define current incidence trends and outcomes for pediatric patients with neuroblastoma. The SEER registry was queried from 1973 to 2002. Overall, 1,646 patients with neuroblastoma were identified. The annual incidence has remained unchanged at 0.9 per 100,000. The median age of the population was 1 year, with 42% of patients presenting at <1 year of age. The majority of tumors arose in the retroperitoneum (75.6%) with the remainder located in the mediastinum (15.3%), cervical region (6.6%) and pelvis (2.2%). Markedly improved survival has been noted in each decade (P < 0.002). Sixteen percent of lesions were over 10 cm in greatest dimension, while 84% were high-grade. Disease-specific survival at 1, 2, 5 and 20 years for the entire cohort was 81, 70, 61 and 59%, respectively. Superior survival was observed for infants <1 year of age (P < 0.001). Neuroblastomas in the mediastinum and pelvis had a better prognosis (P < 0.05) while high-grade and lesions over 10 cm carried a worse prognosis (P < 0.022). Surgery but not radiotherapy was associated with improvement in survival (P < 0.001). Multivariate analysis identified age, tumor location, stage, decade of diagnosis and surgical treatment as independent prognostic factors. Neuroblastoma remains a common malignancy with markedly improving patient outcomes. Early diagnosis and surgical therapy continue to provide the best chance for cure. More effective therapies for patients presenting over 1 year of age or those with advanced disease are still needed.
我们试图明确神经母细胞瘤儿科患者当前的发病率趋势及预后情况。对监测、流行病学与最终结果（SEER）数据库1973年至2002年的数据进行了查询。总体而言，共识别出1646例神经母细胞瘤患者。年发病率保持不变，为每10万人中有0.9例。患者的中位年龄为1岁，42%的患者发病年龄小于1岁。大多数肿瘤发生于腹膜后（75.6%），其余位于纵隔（15.3%）、颈部区域（6.6%）和骨盆（2.2%）。每十年生存率均有显著提高（P < 0.002）。16%的病灶最大直径超过10 cm，84%为高级别。整个队列1年、2年、5年和20年的疾病特异性生存率分别为81%、70%、61%和59%。1岁以下婴儿的生存率更高（P < 0.001）。纵隔和骨盆的神经母细胞瘤预后较好（P < 0.05），而高级别和直径超过10 cm的病灶预后较差（P < 0.022）。手术而非放疗与生存率提高相关（P < 0.001）。多变量分析确定年龄、肿瘤位置、分期、诊断年代和手术治疗为独立的预后因素。神经母细胞瘤仍然是一种常见的恶性肿瘤，患者预后显著改善。早期诊断和手术治疗仍然是治愈的最佳机会。对于1岁以上或患有晚期疾病的患者，仍需要更有效的治疗方法。
