Introduction
The aim of the study was to analyze the prognostic factors in the cohort of patients with low-risk neuroblastoma (NB) treated in Russian Federation.
Methods
157 patients with low-risk NB were treated for the period 01.2012-06.2015 (42 months). The diagnosis has been established on the basis of the international criteria. Patients were stratified and treated according to the German NB2004 protocol. Surgery was the mainstay of therapy. Chemotherapy was reserved for the patients with life-threatening symptoms (LFS) persisted after surgery.
Results
Male: female ratio was – 1.04:1. The median age at the diagnosis was 5.8 months (range 0.5- 205.4). 34 (21.7%) patients had LFS at the time of diagnosis. 1p and 11q deletion was observed in 9 (5.7%) and 6 (3.8%) patients, respectively. Initial therapy consisted of surgery in 115 (73.2%), chemotherapy – 27 (17.2%) and observation – 15 (9.5%). Only 35 (22.3%) patients received chemotherapy as a part of first-line therapy. Median follow-up was 24.4 months (range 0.0-59.4). 3-year event-free survival (EFS) and overall survival (OS) was 80.6±3.2% and 96.1±1.5%, respectively. Progression/relapse was observed in 24 (15.2%) patients with a median time to event 4.1 months (range 0.5-17.3). OS after relapse/progression was 95,4±4,4%. 6 (3.8%) patients died. Median time to death was 2.0 months (range 0.0-6.7). 5 deaths were therapy-related, 1 – tumor-related. Cumulative incidence of treatment-related and disease-related mortality at 12 months was 3,2% and 0,6%. Age < 12 months and stage 3 were associated with inferior EFS; non-abdominal location, presence of LFS and stage 3 were associated with inferior OS in the univariate analyzes. 1p and 11q status didn’t affect prognosis. In multivariate analyzes only presence of LFS associated with OS.
Conclusion
The prognosis for patients with low-risk NB is good. Risks associated with therapy should be considered in patients with LFS when choosing treatment strategies.