Thoracic Neuroblastic Tumors in Childhood

被引:18
作者
Demir, Haci Ahmet [1 ]
Yalcin, Bilgehan [1 ]
Buyukpamukcu, Nebil [2 ]
Kale, Guelsev [3 ]
Varan, Ali [1 ]
Akyuz, Canan [1 ]
Kutluk, Tezer [1 ]
Buyukpamukcu, Munevver [1 ]
机构
[1] Hacettepe Univ, Dept Pediat Oncol, Inst Oncol, TR-06100 Ankara, Turkey
[2] Hacettepe Univ, Fac Med, Dept Pediat Surg, TR-06100 Ankara, Turkey
[3] Hacettepe Univ, Fac Med, Dept Pediat Pathol, TR-06100 Ankara, Turkey
关键词
children; neurogenic tumors; posterior mediastinum; thoracic neuroblastomas; PRIMARY MEDIASTINAL TUMORS; NEUROGENIC TUMORS; JAPANESE INSTITUTION; PEDIATRIC-ONCOLOGY; EXPERIENCE; CHILDREN; MASSES;
D O I
10.1002/pbc.22405
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
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. Pediatr Blood Cancer 2010;54:885-889. (C) 2010 Wiley-Liss, Inc.
引用
收藏
页码:885 / 889
页数:5
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