Treatment and outcomes of high-risk neuroblastoma in Southeast Asia: a single institution experience and review of the literature

Abstract

Introduction: In Europe and Northern America, the majority of children with high-risk neuroblastoma survive the disease. Elsewhere, treatment outcomes are poor.

Methods: A retrospective review of children treated for high-risk neuroblastoma in a single institution in Singapore from 2007 to 2019 was carried out. Treatment consisted of intensive chemotherapy, surgery aimed for gross total resection of residual disease after chemotherapy, consolidation with high-dose therapy followed by autologous stem cell rescue, radiotherapy to the primary and metastatic sites, followed by maintenance treatment with either cis-retinoic acid or anti-disialoganglioside (anti-GD2) monoclonoal antibody therapy. Survival data were examined on certain clinical and laboratory factors. Results: There were 57 children with 32 males treated for high-risk neuroblastoma. Their mean age was 3.9 (0.7–14.9) years. The median follow-up time was 5.5 (1.8–13.0) years for the surviving patients. There were 31 survivors with 27 surviving in first remission, and 5-year overall survival and event-free survival rates were 52.5% and 47.4%, respectively. On log-rank testing, only the group of 17 patients who were exclusively treated at our centre had a survival advantage. Their 5-year overall survival rate, compared with others whose initial chemotherapy was done elsewhere, was 81.6% versus 41.1% (p = 0.011), and that of event-free survival was 69.7% versus 36.1% (p = 0.032). Published treatment results were found from four countries in Southeast Asia with 5-year overall survival rates from 13.5%–28.2%.

Conclusion: Intensified medical and surgical treatment for high-risk neuroblastoma proved to be effective with superior survival rates compared with previous data from Southeast Asia.