Parise Ivy Zortéa S, Haddad Bassem R, Cavalli Luciane R, Pianovski Mara A D, Maggio Ewerton M, Parise Guilherme A, Watanabe Flora M, Ioshii Sérgio O, Rone Janice D, Caleffe Luiz Gonzaga, Odone Filho Vicente, Figueiredo Bonald C
Division of Pediatric Hematology and Oncology, Hospital de Clínicas, Federal University of Paraná, Brazil.
J Pediatr Hematol Oncol. 2006 Feb;28(2):82-7. doi: 10.1097/01.mph.0000199601.35010.52.
The authors report on the incidence and clinical characteristics of neuroblastoma in southern Brazil. The aims of the study were to evaluate the age at diagnosis, tumor stage, MYCN status, and tumor histopathology, and to relate these factors to survival. All patients with neuroblastoma, 15 years old or younger (n = 125), admitted to the three major pediatric oncology hospitals in the state of Parana over a period of 11 years (between January 1990 and December 2000), were included in the analysis. All patients were followed for at least 5 years. In addition, a FISH evaluation for MYCN status was conducted in a subset of 34 tumors. Overall survival for tumor stages 1, 2, 3, and 4 was 100%, 72%, 59%, and 17%, respectively. Sixty-two percent (77/125) of all patients were older than 2 years; these represented 71% (57/80) of the patients with stage 4 disease. Children who presented with an unfavorable histopathology had a significantly worse prognosis (20% survival) than children with a favorable histopathology (67% survival). MYCN amplification was detected most commonly in stages 3 and 4 tumors (13/16). These data showed a delayed diagnosis of neuroblastoma in children in southern Brazil, and consequently survival was considerably lower in these patients.
作者报告了巴西南部神经母细胞瘤的发病率及临床特征。本研究的目的是评估诊断时的年龄、肿瘤分期、MYCN状态和肿瘤组织病理学，并将这些因素与生存率相关联。纳入分析的对象为11年间（1990年1月至2000年12月）入住巴拉那州三家主要儿科肿瘤医院的所有15岁及以下的神经母细胞瘤患者（n = 125）。所有患者均随访至少5年。此外，对34个肿瘤的子集进行了MYCN状态的荧光原位杂交（FISH）评估。肿瘤1期、2期、3期和4期的总生存率分别为100%、72%、59%和17%。所有患者中有62%（77/125）年龄超过2岁；这些患者占4期疾病患者的71%（57/80）。组织病理学不良的儿童预后明显比组织病理学良好的儿童差（生存率分别为20%和67%）。MYCN扩增最常见于3期和4期肿瘤（13/16）。这些数据表明巴西南部儿童神经母细胞瘤诊断延迟，因此这些患者的生存率相当低。
