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Thoracic neuroblastic tumors in childhood

Authors :
Haci Ahmet, Demir
Bilgehan, Yalçin
Nebil, Büyükpamukçu
Gülsev, Kale
Ali, Varan
Canan, Akyüz
Tezer, Kutluk
Münevver, Büyükpamukçu
Source :
Pediatric bloodcancer. 54(7)
Publication Year :
2010

Abstract

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.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.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).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.

Details

ISSN :
15455017
Volume :
54
Issue :
7
Database :
OpenAIRE
Journal :
Pediatric bloodcancer
Accession number :
edsair.pmid..........8d10bdfa7755b17a6e7f260a0391691a