Ciência e terapia do câncer

Non Metastatic Rhabdomyosarcoma in Children: Therapeutic Outcome and Prognostic Factors of a Single Institution Case Series

Abstract

Feryel Letaief Ksontini

Background: Rhabdomyosarcoma (RMS) is the most common soft tissue tumor in childhood with a cure rate of 70% in localized disease. We aimed to study the prognostic factors of non-metastatic RMS in Tunisian paediatric patients.

Methods: We reviewed data of children aged <18 years, treated in the Salah Azaiez Institute for a localized RMS during 20 years. Prognostic factors were studied and survival data analysed.

Results: 75 patients were included. Mean follow up was 30 months. The 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. The surgery failed to be a significant factor. We didn`t find any factor with a significant relationship with DFS 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. Age, histology, primary site and IRS group failed to be significant. By multivariate analysis, OS was strongly correlated to radiotherapy p=0.03, Odds Ratio (OR) 3.1, confidence interval (IC) 95% [1.05-9.3] and para-meningeal site p=0.04, (OR) 0.3, confidence interval (IC) 95% [0.1-0.9].

Conclusions: Compared to the literature, we noticed that the prognosis of our patients was worse so we should improve it by making the treatment more personalized and encouraging research.