Treatment of relapse/progression of the disease in patients with neuroblastoma in the Republic of Belarus over a 20-year period: a cohort study

Abstract

Introduction. Neuroblastoma (NB) — is a malignant tumor of sympathetic nervous system in children. In our country there are about 50 % of patients in high risk group, 50 % have a relapse or a progression of the disease which is the main reason for their death. The purpose of the study . Our aim was to characterize a relapse/progression of NB in children in the Republic of Belarus, the analysis of results of treatment of patients from 1997 till 2017. Materials and methods . From October, 1997 to December, 2017 in Republican Scientific and Practical Center for Pediatric Oncology, Hematology and Immunology 293 protocol and observed patients received 1st line therapy. Out of them 73 patients had a progression or a relapse of the disease. This group of patients was taken for the analysis. Results. 14-year overal survival of patients with a relapse/progression of the disease was 21 + 6 % (n = 73), event free survival 18 + 5 % (n = 53). Patients older than 12 months at the time of diagnosis were statistically significantly more likely to recur, with 4th stage disease and having N-MYC amplification, diploid karyotype in the tumor. Adverse relapse options for localization are about 65 % (generalized — 41 %, osteomedullary — 12.3 %, relapses with CNS damage — 12.3 %). The most curable variants of relapses are found in 35 % (n = 25) of cases. In patients from the group of favorable and intermediate prognosis, relapses in the zone of primary tumor localization were significantly more frequent (p < 0.0001), bone loss in the relapse of the disease was significantly less common (p < 0.0001). The best results with the use of standard therapies are relapse with the primary tumor zone and soft tissue relapse (by 5 year relapse-free survival — 25% and 36 % respectively). Treatment of osteomedullary and generalized relapses using the methods available to us is not effective (EFS is 0). Conclusion. Further detailed study of the biology of the tumor is necessary, in order to create for the therapy of the disease targeted drugs and other biologically based approaches, such various types of immunotherapy.