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Published on: February 6, 2019
Thoracic neuroblastic tumors in childhood
Haci Ahmet Demir1, Bilgehan Yalçin, Nebil Büyükpamukçu
1Department of Pediatric Oncology, Institute of Oncology, Hacettepe University, Ankara, Turkey. hdemir@hacettepe.edu.tr
Pediatric Blood & Cancer
|January 6, 2010
Summary
Thoracic neuroblastic tumors (NBTs) in children show a favorable prognosis, with survival rates influenced by stage but not age. Earlier diagnosis may contribute to better outcomes for these posterior mediastinal tumors.
Area of Science:
- Pediatric Oncology
- Thoracic Surgery
- Tumor Biology
Background:
- Thoracic neuroblastic tumors (NBTs) are a group of rare pediatric neoplasms.
- These tumors are often associated with a better prognosis compared to other neuroblastic tumor locations.
Purpose of the Study:
- To review the clinical characteristics, treatment outcomes, and prognosis of pediatric patients diagnosed with thoracic NBTs.
- To evaluate the impact of age and tumor stage on survival rates.
Main Methods:
- Retrospective review of medical records for 87 children treated for thoracic NBT between 1973 and 2007.
- Analysis of clinical presentation, pathological subtypes (ganglioneuroma, ganglioneuroblastoma, neuroblastoma), tumor staging, and treatment data.
Main Results:
- Most tumors (86/87) were located in the posterior mediastinum or chest wall.
- Ganglioneuromas (15), ganglioneuroblastomas (26), and neuroblastomas (46) were identified.
- Ten-year overall and event-free survival rates were 71.2% and 67.4%, respectively. Survival was significantly better for stages I, II, and IVS (88.8%) compared to stage III (65.3%) and stage IV (27.8%) (P = 0.0002).
- Symptomatic spinal cord compression (25.3%) and Horner syndrome (17.3%) were observed.
Conclusions:
- Thoracic neuroblastic tumors exhibit a favorable prognosis in pediatric patients.
- Earlier diagnosis and potentially distinct biological characteristics may contribute to improved outcomes.
- Less aggressive treatment strategies may be considered, warranting further research into the biological factors influencing NBT outcomes in the thoracic region.
