The importance of local control management in high-risk neuroblastoma in South Africa

Pediatr Surg Int. 2020 Apr;36(4):457-469. doi: 10.1007/s00383-020-04627-x. Epub 2020 Feb 28.

Abstract

Purpose: To investigate the impact of local therapies on high-risk neuroblastoma (HR-NB) outcomes in South Africa.

Methods: Data from 295 patients with HR-NB from nine pediatric oncology units between 2000 and 2014 were analysed. All patients received chemotherapy. Five-year overall (OS) and event free survival (EFS) were determined for patients who had received local therapy, either surgery or radiotherapy or both.

Results: Surgery was performed in only 35.9% (n = 106/295) patients. Surgical excision was done for 34.8% (n = 85/244) of abdominal primaries, 50.0% (n = 11/22) of thoracic primaries; 22.2% (n = 2/9) neck primaries and 66.7% (n = 8/12) of the paraspinal primaries. Only 15.9% (n = 47/295) of all patients received radiotherapy. Children, who had surgery, had an improved five-year OS of 32.1% versus 5.9% without surgery (p < 0.001). Completely resected disease had a five-year OS of 30.5%, incomplete resections 31.4% versus no surgery 6.0% (p < 0.001). Radiated patients had a five-year OS of 21.3% versus 14.2% without radiotherapy (p < 0.001). Patients who received radiotherapy without surgical interventions, had a marginally better five-year OS of 12.5% as opposed to 5.4% (p < 0.001). Patients who underwent surgery had a longer mean overall survival of 60.9 months, while patients, who were irradiated, had a longer mean overall survival of 7.9 months (p < 0.001). On multivariate analysis, complete metastatic remission (p < 0.001), surgical status (p = 0.027), and radiotherapy status (p = 0.040) were significant predictive factors in abdominal primaries.

Conclusion: Surgery and radiotherapy significantly improve outcomes regardless of the primary tumor site, emphasizing the importance of local control in neuroblastoma.

Keywords: High-risk; Intermediate-risk; Local therapies; Neuroblastoma; Radiotherapy; South Africa; Surgery.

MeSH terms

  • Adolescent
  • Biopsy
  • Child
  • Child, Preschool
  • Combined Modality Therapy / methods
  • Disease-Free Survival
  • Female
  • Humans
  • Incidence
  • Infant
  • Magnetic Resonance Imaging
  • Male
  • Neoplasm Staging*
  • Nervous System Neoplasms / diagnosis
  • Nervous System Neoplasms / epidemiology
  • Nervous System Neoplasms / therapy*
  • Neuroblastoma / diagnosis
  • Neuroblastoma / epidemiology
  • Neuroblastoma / therapy*
  • South Africa / epidemiology
  • Survival Rate / trends
  • Tomography, X-Ray Computed