Lucena Januária Nunes, Alves Maria Teresa Seixas, Abib Simone Campos Vieira, Souza Gabriel Oliveira de, Neves Regina Pukenis de Castro, Caran Eliana Maria Monteiro
Universidade Federal de São Paulo, São Paulo, SP, Brasil.
Rev Paul Pediatr. 2018 Jul-Sep;36(3):254-260. doi: 10.1590/1984-0462/;2018;36;3;00007. Epub 2018 Jul 10.
To describe the clinical and epidemiological characteristics and survival outcomes of children with neuroblastoma (NB) treated at a pediatric oncology center from 1991 to 2012.
A retrospective study with clinical and epidemiological data from 258 patients with neuroblastoma treated at a pediatric oncology center from 1991 to 2012, using medical records.
The average age of the children at diagnosis was 40.5±46.4 months with a median age of 28.9 months (interquartile range 42.2). The male:female ratio was 1.3:1, and 1% of the patients were asymptomatic. The most frequent manifestations were: fever (25%), abdominal pain (22%), abdominal mass (19%), and bone pain (19%). The mean time from symptom onset to diagnosis was 3.0±4.8 months. The most common location of the tumor was the abdomen (63%). Metastases occurred in the bone marrow (37%) and in the bone (33%). Overall survival (OS) and event-free survival (EFS) in five years were 62 and 52%, respectively. The main cause of death was the progression of the disease (72%).
The clinical features of children with neuroblastoma are variable and mostly nonspecific, which makes clinical recognition difficult and, in general, too late. In children less than 5 years old, with an abdominal mass and/or bone pain, irritability, and a fever from an unknown cause, neuroblastoma should be considered as a possible diagnosis.
描述1991年至2012年在一家儿科肿瘤中心接受治疗的神经母细胞瘤(NB)患儿的临床和流行病学特征以及生存结局。
一项回顾性研究,利用病历资料分析了1991年至2012年在一家儿科肿瘤中心接受治疗的258例神经母细胞瘤患者的临床和流行病学数据。
患儿诊断时的平均年龄为40.5±46.4个月,中位年龄为28.9个月(四分位间距为42.2)。男女比例为1.3:1,1%的患者无症状。最常见的表现为:发热(25%)、腹痛(22%)、腹部肿块(19%)和骨痛(19%)。从症状出现到诊断的平均时间为3.0±4.8个月。肿瘤最常见的部位是腹部(63%)。转移发生在骨髓(37%)和骨骼(33%)。五年总生存率(OS)和无事件生存率(EFS)分别为62%和52%。主要死亡原因是疾病进展(72%)。
神经母细胞瘤患儿的临床特征多样且大多无特异性,这使得临床识别困难,总体而言诊断往往过晚。对于5岁以下、有腹部肿块和/或骨痛、烦躁不安以及不明原因发热的患儿,应考虑神经母细胞瘤作为可能的诊断。