Clinical features and efficacy of recent treatment analysis of multimodality treatment for 91 children with neuroblastoma

Abstract

Objective To summarize clinical features of neuroblastoma (NB) in children, and analyze the relationship between age, international neuroblastoma staging system(INSS), tumor biological factors and the efficacy of recent treatment.The significance of multidisciplinary treatment in NB was investigated in order to improve their survi-val rates and life qualities. Methods According to neuroblastoma protocol in Beijing Children's Hospital, Capital Me-dical University (BCH-NB-2007 protocol, based on Hong Kong NB protocol), the patients were divided into 3 groups of low-risk (LR) group, medium risk (MR) group and high-risk (HR) group.All children were followed up to May 31, 2012. Results Total 91 children with NB, 59 boys, 32 girls, median age 37 months(4.5-192.0 months); 69 cases of INSS-Ⅳ and 10 cases of Ⅲ, 56 cases of retroperitoneal and pelvic tumor.Bone marrow metastasis in 53 cases (58.2%), tumor cells 4.5%-100.0%, 56 cases of bone metastases (61.5%), most of them were multiple bone metastases, 21 cases of distant lymph node metastasis (23.0%), 13 cases of liver metastases (14.3%), 5 cases of central nervous system metastases (5.5%). Of 72 patients with fully known biologic features, 45 cases of NB or NB chemotherapy change, 26 cases of ganglioneuroblastoma, only one for ganglioneuroma.Fifty-eight cases of NB had the N-myc gene copies by FISH, 43 cases had N-myc gene gains, 4 cases had N-myc amplification , the average of N-myc gene copies of 58 cases was 5.96±7.81.Total 70 cases for treatment, among 7 cases of LR, 2 cases of INSS-Ⅳs, followed up for 51-53 months, all were in partial remission, and 1 case give up treatment, 4 cases were in complete remission(CR). Eleven of MR were followed up for 31 months, 5 children were CR.Among 52 cases of HR, median follow-up for 33 (4-60) months, 22 cases occurred event (42.3%), 5 cases were relapsed after stopped treatment for median 20 (8-40) months, 15 patients for tumor progression.Using Kaplan-Meier analysis showed the expected 5-year overall survival rate was 64.3%, HR was 55.8%. Conclusions NB is progressive disease.The most common metastasis are bone and bone marrow.Children with metastasis have poor prognosis.The most important role is the intensive chemotherapy-based multi-modality treatment.The N-myc gene copies in NB is concerned with the rapid growth and the poor prognosis.And NB children should be followed up regularly after stopping treatment for 3-5 years, in order to detect any suspicious residual disease.When necessary, to give chemotherapy may further improve survival. Key words: Neuroblastoma; Clinical analysis; Multimodality treatment