Background: Pediatric appendicular bone sarcomas, including osteosarcoma and Ewing's sarcoma, are rare but aggressive malignancies that have a profound impact on survival, physical function, and quality of life (QoL). Treatment options usually involve either limb-salvage surgery (LSS) or amputation (AMP), although evidence about outcomes among these approaches is still limited.
Methods: A retrospective cohort study was conducted involving pediatric cases treated for appendicular bone sarcomas between 2000 and 2021 at the Instituto Português de Oncologia de Lisboa (IPOL), a Portuguese oncology center in Lisbon. It evaluated functional and QoL outcomes and survival. Patients were stratified by surgical approach (LSS vs. AMP) and evaluated by validated outcome tools, including the Toronto Extremity Salvage Score (TESS) and the Medical Outcomes Study Short-Form 36 version 2 (MOS SF-36v2) QoL questionnaire. P-values <0.05 were considered significant.
Results: A total of 62 patients were included, with an overall five-year survival rate of 38%. Poorer survival outcomes were recorded among those presenting with metastatic disease, larger tumor sizes (>8 cm), and those who underwent AMP. Limb-salvage surgery was performed in 59.7% of cases and presented higher mean functional scores (88.4%) compared with AMP (79%). The QoL scores varied, with LSS patients reporting better outcomes in physical and emotional domains. Patients from Portuguese-speaking African countries (PSAC) had poorer survival rates, a finding that reflects disparities that could be related to advanced disease presentation and limited health resources.
Conclusion: Limb-salvage surgery had better functional outcomes and QoL compared to AMP. However, survival of these patients remains a challenge, especially for those from resource-limited settings. These results highlight the need for early diagnosis, improvement in access to healthcare, and further research to improve treatment.
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| http://dx.doi.org/10.7759/cureus.78543 | DOI Listing |
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