Management outcomes of South African children diagnosed with neuroblastoma in adolescence

Abstract

Background

Neuroblastoma (NB) is an indolent disease in adolescents. Only 5 % of neuroblastoma diagnoses are made in adolescents and adults. Limited data exists on Africans of older age groups.

Objectives

We aimed to compare the characteristics and outcomes of South African children ≥10 years diagnosed with NB with international reports.

Patients and methods

Multicentric, retrospective data of South African patients (0–15 y) with NB diagnosed between January 2000 and December 2016 were analyzed. Clinical profiles and outcomes of adolescents (≥10 years) with NB were compared to those of the younger patients in our cohort. The outcomes of adolescents (≥10 years) with NB were compared with those reported in the global literature.

Results

Thirty adolescents with a male-to-female ratio of 1:0.6 were diagnosed with NB, constituting 6.5 % of all patients with NB. Metastatic disease at diagnosis was present in 24 (75 %), including lung metastases in 2 (6 %). Half of the patients presented with a raised lactate dehydrogenase (LDH) and/or ferritin and unfavorable pathology.

Post-induction remission rates were higher in the adolescent group (33 %) compared to the 18–59 months (24.8 %) and 5–9.9 years (29.7 %) cohorts but less than the <18 months (47.2 %) cohort (p < 0.001). The adolescent group had the poorest five-year overall survival (20.9 %) compared to the children <18 months (39.0 %), 18–59 months (44.1 %), or 5–9.9 years old (63.5 %) (p < 0.001).

Conclusions

Despite a favorable response rate to induction chemotherapy, the five-year overall survival of adolescents with NB is dismal in South Africa.