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Malignant Extracranial Germ Cell Tumours: A First Report by the South African Children’s Cancer Study Group
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  • Marc Hendricks,
  • Annibale Cois,
  • Jennifer Geel,
  • Jan du Plessis,
  • Mairi Bassingthwaighte,
  • Gita Naidu,
  • Biance Rowe,
  • Ane Buchner,
  • Fareed Omar,
  • Karla Thomas,
  • Ronelle Uys,
  • Anel van Zyl,
  • Jaques van Heerden,
  • Ngoakoana Mahlachana,
  • Johani Vermeulen,
  • Alan Davidson,
  • Lindsay Frazier,
  • Kirsty Donald,
  • Mariana Kruger
Marc Hendricks
University of Cape Town
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Annibale Cois
Stellenbosch University
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Jennifer Geel
University of the Witwatersrand
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Jan du Plessis
University of the Free State Faculty of Health Sciences
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Mairi Bassingthwaighte
Division of Paediatric Haematology Oncology, Chris Hani Baragwanath Academic Hospital, University of Witwatersrand, Soweto, South Africa
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Gita Naidu
Chris Hani Baragwanath Hospital
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Biance Rowe
Division of Paediatric Haematology Oncology, Chris Hani Baragwanath Academic Hospital, University of Witwatersrand, Soweto, South Africa.
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Ane Buchner
University of Pretoria
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Fareed Omar
Steve Biko Academic Hospital and the University of Pretoria
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Karla Thomas
Paediatric Haematology Oncology, Department of Paediatrics and Child Health, Frere Hospital, East
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Ronelle Uys
Stellenbosch University Faculty of Medicine and Health Sciences
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Anel van Zyl
Stellenbosch University Faculty of Medicine and Health Sciences
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Jaques van Heerden
Paediatric Haematology and Oncology, Department of Paediatrics and Child Health, University of Antwerp, Antwerp University Hospital, Antwerp, Belgium
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Ngoakoana Mahlachana
University of the Witwatersrand
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Johani Vermeulen
Paediatric Haematology Oncology, Department of Paediatrics and Child Health, Port Elizabeth Provincial Hospital, Walter Sisulu University, Port Elizabeth, South Africa
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Alan Davidson
Red Cross War Memorial Children's Hospital and the University of Cape Town
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Lindsay Frazier
Dana Farber Cancer Institute
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Kirsty Donald
Division of Developmental Paediatrics, Department of Paediatrics and Child Health, Red Cross War Memorial Children’s Hospital, and the Neuroscience Institute, University of Cape Town, Cape Town, South Africa.
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Mariana Kruger
Tygerberg Hospital and Stellenbosch University
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Abstract

OBJECTIVE  To determine the overall survival (OS) and prognostic factors influencing outcomes in children and adolescents with malignant extracranial germ cell tumours (MEGCTs) in preparation for the development of a harmonised national treatment protocol.METHODS A retrospective folder review was undertaken at nine South African paediatric oncology units to document patient profiles, tumour and treatment-related data and outcomes for all children with biopsy proven MEGCTs from birth up to and including 16 years of age. RESULTS Between 1 January 2000 and 31 December 2015, 218 patients were diagnosed with MEGCTs. Female sex (HR 0.284 p=0.037) and higher socio-economic status (SES) (HR 0.071; p=0.039) were associated with a significantly lower risk of death. Advanced clinical stage at diagnosis significantly affected 5-year OS: stage I -96%; stage II - 94.3%; stage III -75.5%; (p=0.017) and stage IV (60.1%; p<0.001). There was a significant association between earlier stage at presentation and higher SES (p=0.03). Patients with a serum AFP level of more than 33,000 ng/ml at diagnosis had significantly poorer outcomes (p=0.002). The use of chemotherapy significantly improved survival, irrespective of the regimen used (p<0.001). CONCLUSIONS The cohort demonstrated a 5-year OS of 80.3% with an EFS of 75.3%. Stage, the use of chemotherapy and an elevated serum AFP level of more than 33,000ng/ml were independently predictive of outcome. The relationship between SES and outcome is important as the implementation of the new national protocol hopes to standardise care across the socio-economic divide. 
30 Sep 2021Submitted to Pediatric Blood & Cancer
30 Sep 2021Submission Checks Completed
30 Sep 2021Assigned to Editor
04 Oct 2021Reviewer(s) Assigned
16 Oct 2021Review(s) Completed, Editorial Evaluation Pending
18 Oct 2021Editorial Decision: Revise Major
24 Nov 2021Submission Checks Completed
24 Nov 2021Assigned to Editor
24 Nov 20211st Revision Received
29 Nov 2021Reviewer(s) Assigned
30 Nov 2021Review(s) Completed, Editorial Evaluation Pending
03 Dec 2021Editorial Decision: Accept
May 2022Published in Pediatric Blood & Cancer volume 69 issue 5. 10.1002/pbc.29543