Paediatric bone and soft-tissue sarcomas in Ireland: Epidemiology, management and outcomes.
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BACKGROUND: Paediatric bone and soft tissue sarcomas are rare malignancies, that typically require multimodal oncologic therapy and technically demanding surgery delivered within a specialist multidisciplinary team setting. The epidemiology, management and outcomes of paediatric sarcoma in Ireland have not yet been described. METHODS: This was an observational study of a consecutive cohort of patients aged <16 years with primary bone or soft-tissue sarcoma managed by Ireland's national sarcoma service over a 10-year period. The epidemiology, management, and outcomes of these patients were reported. The primary outcome was overall survival which was reported using Kaplan-Meier estimates. The factors associated with survival were also analysed. RESULTS: Eighty-eight patients were included, of whom 53.4% (47/88) were male. Osteosarcoma was the most common diagnosis (46.6%, 41/88), followed by Ewing's sarcoma (25%, 22/88). Soft-tissue sarcoma accounted for 25% (22/88). Primary sites most frequently involved the femur (33%, 29/88), tibia (18.2%, 16/88), humerus (15.9%, 14/88) and pelvis (11.4%, 10/88). Metastatic pulmonary disease was present at diagnosis in 33% (29/88) of patients. Seventy-six patients underwent surgery. Limb-salvage endoprosthetic reconstruction was most common at 46.1% (35/76), followed by en bloc tumour resection in 36.8% (28/76) and amputation in 17.1% (13/76). All patients with a diagnosis of primary bone sarcoma received multi-modal chemotherapy guided by a multidisciplinary team. Overall mortality rate was 25% (22/88). Estimated 5-year survivorship was 81.5% for osteosarcoma, 93.3% for Ewing sarcoma, and 90.9% for soft-tissue sarcoma. Mortality was highest when metastatic disease was present at the time of diagnosis (51.7% vs 11.9%, p < .001), (HR 2.81, 95% CI 0.96-8.25; p = .061). CONCLUSIONS: The incidence of paediatric sarcoma in Ireland aligns with other European countries. Overall survivorship was relatively high, with metastatic disease at the time of diagnosis associated with lower survivorship. This study provides a benchmark for the current epidemiology and outcomes for children with sarcoma in Ireland.