Malignant Extracranial Germ Cell Tumours: A First Report by the South African Children’s Cancer Study Group

Autor: 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
Rok vydání: 2021
Popis: 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%; pCONCLUSIONS 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.
Databáze: OpenAIRE