Publication
Title
Extracranial germ cell tumours: mature and immature (1990-2015) : first report by the South African Association of Paediatric Haematology Oncology (SAAPHO)
Author
Abstract
Background and objectives: Outcomes of rare paediatric teratomas have not previously been reported nor treatment regimens standardised in low- and middle-income settings. We sought to evaluate treatment outcomes of children and adolescents with histologically confirmed extracranial germ cell tumours, both mature teratomas (MT) and immature teratomas (IT) in preparation for the development of the South African national treatment guideline.Methods: Retrospective data by folder review were collated from nine South African paediatric oncology units. Kaplan-Meier analysis with Cox regression was performed to determine 5-year overall survival (OS) and prognostic factors.Results: From January 1990 to December 2015, 60 patients were diagnosed with MTs; 14 males (median age 2 months; interquartile range [IQR]: 0-8.75 months) and 46 females (median age 9 months; IQR: 0-88.5 months). Forty patients were diagnosed with ITs; 10 males (median age less than 1 month; IQR: 0-1.75 months) and 30 females (median age 4.5 months; IQR: 1-162 months). There were high rates of upfront surgical resections in patients with MTs (58/60; 96.6%) and ITs (36/40; 90%), and similarly satisfactory rates of complete resection in patients with both MTs (55/60; 91.7%) and ITs (32/40; 80%). The 5-year OS for the whole group was 85.4%, significantly influenced by stage: Stage I (96.9%), Stage II (100%), Stage III (38.9%) (p < .001 [MT]; p = .013 [IT]). The event-free survival (EFS) ratio for the whole cohort was 78.7%.Conclusions: Five-year OS for those with low-stage disease was excellent, but was poorer for patients with advanced disease. The implementation of a national treatment guideline will facilitate the standardising of surgical approaches, indications for chemotherapy and specifications for follow-up to improve survival and to collect more robust late effects data.
Language
English
Source (journal)
Pediatric Blood and Cancer. - -
Publication
Hoboken : Wiley , 2024
ISSN
1545-5009
DOI
10.1002/PBC.30723
Volume/pages
71 :1 (2024) , p. 1-8
Article Reference
e30723
ISI
001089452300001
Pubmed ID
37872125
Full text (Publisher's DOI)
Full text (open access)
UAntwerpen
Faculty/Department
Research group
Publication type
Subject
Affiliation
Publications with a UAntwerp address
External links
Web of Science
Record
Identifier
Creation 04.12.2023
Last edited 07.12.2023
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