Non Metastatic Rhabdomyosarcoma in Children and Adolescents: Prognostic Factors and Survival
Journal Title: Journal of Orthopaedic Science and Research - Year 2021, Vol 2, Issue 3
Abstract
Background: Rhabdomyosarcoma (RMS) is the most common softttissue tumor in childhood. We aimed to study the prognostic factors of non-metastatic RMS in Tunisian paediatric patients. Methods: We reviewed data of paediatric patients (aged < 18 years), with histologically confirmed RMS treated in Salah Azaiez Institute for a non-metastatic RMS during 20 years. Prognostic factors were studied and survival data analysed. Results: 75 patients were included (42 males and 33 females). Embryonal RMS was the most common histologic type (72%) followed by alveolar (21%) and pleomorphic (1%). The most frequently affected sites were head and neck (43%) and genito-urinary (28%). 5-year OS and DFS were 50% and 26%, respectively. By univariate analysis, DFS was significantly correlated to chemotherapy, Radiotherapy (RT) and post-surgical RT with p=0.02, 0.003 and 0.01, respectively. No factor was significant in multivariate analysis. By univariate analysis, 5-year OS was significantly and adversely influenced by 4 factors: tumour size > 4 cm, non-alveolar RMS, positive regional nodes and para-meningeal location, with p=0.050, 0.05, 0.04 and 0.04, respectively. RT and postsurgical RT were associated with a good prognosis in OS p=0.009 and 0.05, respectively. By multivariate analysis, OS was strongly correlated to radiotherapy p=0.03, Odds Ratio (OR) 3.1, (IC) 95% [1.05-9.3] and para-meningeal site p=0.04, (OR) 0.3, (IC) 95% [0.1-0.9]. Conclusions: This study showed that tumor size, histological type, tumor location, node involvement, t CT and RT were prognostic factors for OS and PFS. However, survival remains poor. So we should improve it by encouraging clinical research.
Authors and Affiliations
Feryel Letaief-Ksontini*, Mouna Ayadi, Azza Gabsi, Safia Yahiaoui, Houcine Magherbi, Amina Mokrani, Khedija Meddeb
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