| Objective: The aim of the paper was to provide reference for further optimization of treatment of neuroblastoma through the exploration of the clinical features,therapeutic effect and prognostic factors.Methods: Children younger than 18 years old with neuroblastoma were eligible if they were diagnosed between January 2015 to July 2017.A total of sixty patients were included for the retrospective analysis.The treatment option is the 2015 neuroblastoma expert consensus developed by China Children Cancer Group.Results:(1)A total of 60 patients were included in this study.Among them,30 were male and 30 were female,the onset age ranging from 2.5 months to 140 months,the average age is 34.2 months and the median age is 26.5 months,the percentage of patients under 3 years old was 61.67%.(2)The primary clinical manifestations: 15 cases had fever,10 cases had anemia,12 cases had imaging examination after prolonged coughing,12 cases experienced a physical examination found abdominal mass(all of them were asymptomatic),12 cases experienced abdominal pain or abdominal distension,11 cases suffered from joint pain or stumble,4 cases had neck mass,3 cases had high clinic myocardial enzyme,2 cases suffered from nausea and anorexia.Each of the other 7 cases had periorbital ecchymose,thoracodynia,intussusception,constipation,refractory diarrhea,hypertension,pathological fracture,respectively.(3)Primary tumor site: 11 cases had adrenal tumors,26 cases had retroperitoneal tumors,19 cases had mediastinal tumors,2 cases had pelvic tumors,2 cases had neck tumors.(4)Distant metastasis: 38 cases with distant metastasis,23 cases with bone metastasis,20 cases with bone marrow metastasis,8 cases with liver metastasis,10 cases with renal transfer,8 cases with vertebral canal invasion,3 cases with pancreatic metastasis,2 cases with lung metastasis,16 cases with the lymph node metastasis,1 case with splenic metastasis,20 cases with multiple organic metastasis.(5)A total of 51 patients were treated with pathologic biopsy,34 cases with neuroblastoma tumors,8 cases with ganglio-neuroblastoma intermixed(GNBi)tumors,9 cases with ganglio-neuroblastoma nodular(GNBn)tumors.19 case of which had favorable histology,the rest,32 cases,had unfavorable histology.Furthermore,9 cases underwent bone marrow biopsy before chemotherapy,and no pathological biopsy was performed.(6)The postoperative International Neuroblastoma Staging System(INSS)was carried out in 56 cases: 11 cases in Stage ?,8 cases in Stage?,12 cases in Stage?,25 cases in Stage?.Grade of risk: 15 cases in the low-risk group,13 cases in the intermediate-risk group,30 cases in the high-risk group.(7)The follow-up: 4 cases given up treatment or adherence to medication in other hospital.(8)The treatment result: 55 cases were treated as planned and received effectively follow-up.The follow-up time was 0.5-33 months,the average survival time was 14.6 months,the median survival time was 14 months.To the end of the follow-up time was 2017.07.31,14 cases were under treatment,41 cases had finished the whole Treatment.The overall survival rate was 78.18%,1-year survival rate was 86.00%,2-year survival rate was 63.70%;the survival rate covered stage?was 100%,covered stage ?was 87.50%,covered stage ? was 72.73%,covered stage ? was 68.00%.19 cases(34.55%)achieved complete remission(CR),17 cases(30.90%)achieved very good partial response(VGPR),5 cases(9.00%)achieved partial response(PR),11 cases(20.00%)achieved progressive disease(PD),3 patients(5.45%)achieved mixed response(MR).(9)Single factor analysis showed that prognostic included tumor primary site,LDH(>2 times the high normal range),serum NSE(>100mg/L),amplification of MYCN gene,bone marrow metastasis,bone metastasis,whether complete resection of tumor.(10)Multivariate results showed that independent prognostic factors included amplification of MYCN gene,bone marrow metastasis and whether complete resection of tumor.Conclusion: The pathogenesis of neuroblastoma is concealed,and in almost 50% of the cases the disease is already metastatic at initial diagnosis.It is an extremely heterogeneous disease that can regress spontaneously even without therapy.However,it frequently shows a very aggressive behavior,refractory to current intensive multimodal therapy.Patients with high risk have poor prognosis.Surgical resection,chemotherapy,radiation therapy,stem cell transplantation therapies and other novel therapy should be adopted for the comprehensive treatment.The single factors analyzed significantly affect the prognosis that should be tested routinely and timely.For patients with poor prognostic risk factors,adjust the treatment plan timely is necessarily,individual treatment should adopt timely. |
PDF Full Text Request