ACR Index: 6.3
Thoracic neuroblastoma is a neoplasm arising from sympathetic ganglia and accounts for approximately 16% of all neuroblastomas. These typically present as a posterior mediastinal mass at a median age of 0.9 years. Other tumors arising from sympathetic ganglia include ganglioneuroblastoma and ganglioneuroma, which more commonly occur in older children.
The clinical presentation of thoracic neuroblastoma is characterized by a variety of symptoms and signs, including fever, malaise, back pain, anemia, and respiratory symptoms such as cough and shortness of breath. Apical lesions may produce Horner's syndrome, and spinal canal involvement may cause lower limb weakness. Rarely, patients may present with the opsoclonus cerebellar ataxia syndrome. That being said, approximately 50% of thoracic neuroblastomas are found incidentally on chest films.
Chest radiographs demonstrate a posterior mediastinal mass, which may be mistaken for pneumonia, atelectasis, or normal thymus. Rib erosion and costal separation may be evident. Calcification is often present and metastatic tumors are most often bilateral, paravertebral, and in the lower posterior mediastinum. Computed tomography typically shows an inhomogeneously enhancing paraspinal mass. Approximately 40% of lesions show calcification, and extradural extension occurs in approximately 20%. MRI is valuable in determining intraspinal extension, marrow involvement, and chest wall and lymph node involvement.
Diagnosis is made with biopsy of the lesion, and complete surgical resection is the treatment of choice. Compared to abdominal neuroblastoma, thoracic neuroblastoma presents at an earlier age and stage, and is associated with a more favorable 4-year survival rate (58% versus 48%).
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