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BACKGROUND: The survival rate of children with neuroblastoma has been improved over 20 years, excluding the metastatic disease, in which it does not exceed 20% so far. New treatment modalities have been developed to improve the outcome in metastatic disease. Preoperative chemotherapy reduce the size, the vascularity and the adhesiveness, so increase the resectability of the primary tumor. This retrospective clinical study was designed to review the survival rate in neuroblastoma and to analyze the effect of preoperative chemotherapy in the view point of neoadjuvant therapy on long-term survival in advanced disease. METHODS: One hundred and thirty-four cases were reviewed from 135 patients with neuroblastoma registered at the Department of Pediatrics in Seoul National University Children's Hospital from January, 1985 till December, 1995. The survival rate was reviewed according to the stage. The age, sex of the patients, the stage, anatomical site of the tumor, the level of serum ferritin and neuron-specific enolase were analyzed for the risk factors on survival. RESULTS: Ranges of age at diagnosis were from 1 month to 166 months with the median of 39 months. Five year survival rates and five year disease-free survival rates were 100%, 100% in stage 1(n=5), 90.9%, 90.9% in stage 2(n=13), 43.4%, 40.6% in stage 3(n=19), 27.1%, 19.8% in stage 4(n=95) and 100%, 100% in stage 4S(n=2), respectively. In stage 3, five year survival rate was 52.5% in group receiving neoadjuvant chemotherapy, 28.6% in control group(P=0.02). Five year disease-free survival rate was also noted as 48.6%, 28.6% in each group(P=0.02). In stage 4, five year and ten year survival rates were 27.6%, 23.6% in group receiving neoadjuvant chemotherapy, 26.9%, 0% in control group(P=0.02). Five year and ten year disease-free survival rates were 14.3%, 14.3% in group receiving neoadjuvant chemotherapy, 20%, 0% in control group (P=0.11). In univariate analysis, the age, the stage, and the site of primary tumor appeared to affect the long-term survival. CONCLUSION: Neoadjuvant chemotherapy and delayed primary surgery contribute for advance in survival in advanced neuroblastoma via increasing the resectability of the primary tumor.
Adhesiveness ; Child ; Diagnosis ; Disease-Free Survival ; Drug Therapy* ; Ferritins ; Humans ; Neoadjuvant Therapy ; Neuroblastoma ; Pediatrics ; Phosphopyruvate Hydratase ; Retrospective Studies ; Risk Factors ; Seoul ; Survival Rate

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