Vale Sara S, Castro Rui, Andrade Alexandra, Faleiro Joana, Abreu Nina, Mendes Cristina, Gonçalves Jean-Pierre
Pediatrics, Unidade Local de Saúde Região de Leiria Entidade Pública Empresarial (EPE), Leiria, PRT.
Family Medicine, Unidade Local de Saúde Médio Ave Entidade Pública Empresarial (EPE), Santo Tirso, PRT.
Cureus. 2025 Feb 5;17(2):e78543. doi: 10.7759/cureus.78543. eCollection 2025 Feb.
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.
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.
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.
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.
儿童附肢骨肉瘤,包括骨肉瘤和尤因肉瘤,虽罕见但具有侵袭性,对生存、身体功能和生活质量(QoL)有深远影响。治疗选择通常包括保肢手术(LSS)或截肢(AMP),不过关于这些方法的结果证据仍然有限。
进行了一项回顾性队列研究,纳入2000年至2021年在里斯本的葡萄牙肿瘤研究所(IPOL)接受附肢骨肉瘤治疗的儿科病例。该研究评估了功能和生活质量结果以及生存率。患者按手术方法(LSS与AMP)分层,并通过经过验证的结果工具进行评估,包括多伦多肢体挽救评分(TESS)和医疗结果研究简明健康调查第2版(MOS SF-36v2)生活质量问卷。P值<0.05被认为具有统计学意义。
共纳入62例患者,总体五年生存率为38%。有转移疾病、肿瘤较大(>8 cm)以及接受AMP的患者生存率较差。59.7%的病例进行了保肢手术,与AMP(79%)相比,保肢手术的平均功能评分更高(88.4%)。生活质量评分各不相同,保肢手术患者在身体和情感领域的结果更好。来自非洲葡语国家(PSAC)的患者生存率较低,这一发现反映了可能与疾病进展和卫生资源有限相关的差异。
与截肢相比,保肢手术具有更好的功能结果和生活质量。然而,这些患者的生存仍然是一个挑战,特别是对于那些来自资源有限地区的患者。这些结果凸显了早期诊断、改善医疗服务可及性以及进一步开展研究以改进治疗的必要性。