title: A biochemical analysis of thoracic neuroblastomas: a Pediatric Oncology Group study.
abstract: A biochemical analysis was performed on tumor tissues from 20 patients who presented with thoracic neuroblastomas. Nine patients were under 1 year of age at the time of diagnosis, and 12 patients had stage A disease. Eighteen of the 20 patients are disease free with a mean follow-up of 5 1/2 years. The ganglioside composition of the tumor tissue was investigated, since these cell membrane components have been proposed to play a role in cell to cell interaction and may be altered on cell transformation. In addition, the ganglioside composition of the central nervous system changes with maturation. Previous studies in children with neuroblastoma have shown that tumor tissue containing more complex gangliosides is associated with a better prognosis. Neuroblastomas from patients with thoracic primaries were found to contain more complex gangliosides of the b series (GD1b, GT1b) and fewer monosialogangliosides, suggesting a more differentiated cellular composition. Tissue from one of the thoracic patients who died lacked GT1b. The absence of this ganglioside has proven to be an indicator of a poor prognosis. Four specimens contained no detectable GD2, which is thought to be a specific marker for neuroblastomas. These data suggest that the improved prognosis seen with thoracic neuroblastomas is due to a basic biologic difference within these tumors, and this finding should be taken into consideration when planning therapy.
mesh_terms: D003131:Combined Modality Therapy; D005732:Gangliosides / Q000378:metabolism*; D006801:Humans; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000473:pathology* / Q000601:surgery; D011379:Prognosis; D013899:Thoracic Neoplasms / Q000473:pathology* / Q000601:surgery
chemical_list: D005732:Gangliosides