Neuroblastoma: Can lessons from the past help to improve the future?

Abstract

Background. The outcome of patients with neuroblastoma in South Africa has always been very poor. We conducted a retrospective study in one state-funded paediatric oncology unit (POU), to describe the clinical course, evaluate prognostic factors and report outcomes of patients with neuroblastoma.

Methods. We analysed routine data from one POU, gathered between 1993 and 2018. Kaplan-Meier curves were used to illustrate 2-year survival rates and to evaluate possible prognostic factors.

Results. Data from 87 patients were included and analysed. The median age was 41 months. The majority of the patients presented with stage 4 disease (77%). The most common presenting symptoms were bone pain, loss of weight, and abdominal distention. Chemotherapy was administered to 74 patients, and only 5 patients (6%) received palliative chemotherapy as first-line treatment. Only 18 of the 87 patients had surgery (21%) and 13 of 87 had radiation (15%), while 10 patients received palliative radioactive iodine (131I-miBG) therapy. Patients with ferritin levels >120 ng/dL did not have a poorer outcome, and those with a raised lactate dehydrogenase (LDH) level displayed a shorter survival time but it was not statistically significant. The 2-year overall survival was 24% for the whole cohort and 16% for the stage 4 patients at diagnosis.

Conclusion. Neuroblastoma is a disease with a dismal outcome in our POU, mostly as a result of late presentation. To improve prognosis the focus should be on recognising danger signs to ensure early diagnosis and referral. We recommend adding danger signs for childhood cancer to the Integrated Management of Childhood Illness (IMCI) strategy in an attempt to improve early recognition and diagnosis of childhood cancer.