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

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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.

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Abbreviations

COG:

Children’s Oncology Group

EFS:

Event-free survival

FH:

Favorable histology

GTR:

Gross total resection

HIC:

High-income countries

HR:

High risk

IDRF:

Image define risk factors

INSS:

International Neuroblastoma Staging System

LMIC:

Low- and middle-income countries

NA:

Not amplified

NB:

Neuroblastoma

nGTR:

Near gross total resection

OS:

Overall survival

PFS:

Progression-free survival

PTR:

Primary tumor site relapse

POUs:

Pediatric oncology units

RT:

Radiotherapy

STR:

Subtotal resection

UH:

Unfavorable histology

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Acknowledgements

Dr. van Heerden, as staff member of the Department of Paediatric Haematology and Oncology, Antwerp University Hospital, University of Antwerp, acknowledges the Department for research support. The authors acknowledge the SACCSG, for supporting the study, and the South African Children’s Tumour Registry (SACTR), for providing statistical data.

Author information

Authors and Affiliations

  1. Paediatric Haematology and Oncology, Department of Paediatrics and Child Health, Faculty of Medicine and Health Sciences, Tygerberg Hospital, Stellenbosch University, Cape Town, South Africa

    Jaques van Heerden, Mariana Kruger & Ronelle Uys

  2. Biostatistics Unit, Faculty of Medicine and Health Sciences, Stellenbosch University, Stellenbosch, South Africa

    Tonya Esterhuizen

  3. Department of Paediatrics and Child Health, Faculty of Health Sciences, Paediatric Haematology and Oncology Service, Red Cross War Memorial Children’s Hospital, University of Cape Town, Cape Town, South Africa

    Marc Hendricks

  4. Division of Paediatric Haematology and Oncology, Department of Paediatrics and Child Health, Faculty of Health Sciences, Charlotte Maxeke Johannesburg Academic Hospital, University of the Witwatersrand, Johannesburg, South Africa

    Jennifer Geel

  5. Paediatric Haematology and Oncology, Department of Paediatrics, Steve Biko Academic Hospital, University of Pretoria, Pretoria, South Africa

    Ané Büchner

  6. Division of Paediatric Haematology and Oncology, Department of Paediatrics and Child Health, Faculty of Health Sciences, Chris Hani Baragwanath Academic Hospital, University of the Witwatersrand, Johannesburg, South Africa

    Gita Naidu

  7. Division of Paediatric Haematology and Oncology, Department of Paediatrics, Faculty of Health Sciences, Universitas Hospital, University of the Free State, Bloemfontein, South Africa

    Jan du Plessis

  8. Division of Paediatric Haematology and Oncology Hospital, Department of Paediatrics, Frere Hospital, East London, South Africa

    Barry Vanemmenes

  9. Department of Paediatric Surgery, Faculty of Health Sciences, Nelson R Mandela School of Medicine, University of KwaZulu-Natal, Durban, South Africa

    G. P. Hadley

Authors
  1. Jaques van Heerden
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  2. Mariana Kruger
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  7. Gita Naidu
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  8. Jan du Plessis
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  9. Barry Vanemmenes
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  11. G. P. Hadley
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For the South African Children’s Cancer Study Group

Contributions

JvH conceptualized and designed the study, collected data, performed the data analysis and wrote the manuscript. MK assisted with concept development, as well as design of the study, supervised data analysis, critically reviewed and revised the manuscript. TE performed the statistical analysis. GPH, as expert pediatric surgeon, supervised the surgical content and contributed patient-related data. All other authors collected data in their respective pediatric oncology units and contributed significantly to the manuscript.

Corresponding author

Correspondence to Jaques van Heerden.

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Table 7 INRG classification
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7.

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van Heerden, J., Kruger, M., Esterhuizen, T. et al. The importance of local control management in high-risk neuroblastoma in South Africa. Pediatr Surg Int 36, 457–469 (2020). https://doi.org/10.1007/s00383-020-04627-x

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