Extracranial germ cell tumours: Mature and immature (1990-2015). First report by the South African Association of Paediatric Haematology Oncology (SAAPHO)

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.