儿童胸神经母细胞瘤。

Thoracic neuroblastic tumors in childhood.

机构信息

Department of Pediatric Oncology, Institute of Oncology, Hacettepe University, Ankara, Turkey.

出版信息

Pediatr Blood Cancer. 2010 Jul 1;54(7):885-9. doi: 10.1002/pbc.22405.

DOI:10.1002/pbc.22405

PMID:20049935

Abstract

AIM

Thoracic neuroblastic tumors (NBTs) are reported to have better prognosis. We aimed to review clinical characteristics, treatment results, and outcome of our patients with thoracic NBT.

PATIENTS AND METHODS

Files of 87 children treated at our hospital between 1973 and 2007 with the diagnoses of thoracic NBT were reviewed for clinical and pathological characteristics. Treatment results and outcomes of these cases were examined.

RESULTS

All but one tumors were located in posterior mediastinum, one in the posterior chest wall. Median age of all was 2.1 years (range, 0.03-14; F/M: 1.42). Fifteen cases had ganglioneuromas (GN), 26 ganglioneuroblastomas (GNBL), and 46 neuroblastomas (NBLs). Stages were: I, 20.5%; II, 22.1%; III, 38.2%; IV, 14.7%; IVS, 4.5%. Stages III and IV were more common in cases over 1 year of age. In 20 patients diagnoses were incidental. Twenty-two of 87 (25.3%) had symptomatic spinal cord compression and 15 (17.3%) had Horner syndrome. Ten-year overall and event-free survival rates were 71.2% and 67.4%, respectively. Survival rates did not differ depending on the age being younger or older than 1 year. Ten-year survival rates were 88.8% in stages I, II, IVS; 65.3% in stage III and 27.8% in stage IV (P = 0.0002).

CONCLUSIONS

Thoracic NBLs had a favorable prognosis. This might be a result of earlier diagnosis and some distinct biological characteristics. Favorable prognosis would suggest less aggressive treatment for such patients. Further studies on the biological characteristics of NBLs in the thoracic site and their association with outcome should be done.

摘要

目的

胸神经母细胞瘤（NBT）的预后较好。我们旨在回顾我院 1973 年至 2007 年间诊断为胸 NBT 的 87 例儿童患者的临床特征、治疗结果和结局。

方法

对我院 1973 年至 2007 年间诊断为胸 NBT 的 87 例患儿的病历进行回顾性分析，总结其临床和病理特征。并对这些病例的治疗结果和结局进行分析。

结果

除 1 例位于后胸壁外，其余肿瘤均位于后纵隔。所有患者的中位年龄为 2.1 岁（范围：0.03-14 岁；男女比为 1.42）。15 例为节细胞神经瘤（GN），26 例为节细胞神经母细胞瘤（GNBL），46 例为神经母细胞瘤（NBL）。分期为：Ⅰ期 20.5%；Ⅱ期 22.1%；Ⅲ期 38.2%；Ⅳ期 14.7%；ⅣS 期 4.5%。年龄大于 1 岁的患者中，Ⅲ期和Ⅳ期更为常见。20 例为偶然发现。22 例（25.3%）有症状性脊髓压迫，15 例（17.3%）有霍纳综合征。10 年总生存率和无事件生存率分别为 71.2%和 67.4%。生存率与年龄大小无关。Ⅰ、Ⅱ、ⅣS 期的 10 年生存率分别为 88.8%、65.3%、65.3%，而Ⅲ期和Ⅳ期分别为 65.3%和 27.8%（P=0.0002）。