P0050 Survival of children with rhabdomyosarcoma: Experience of the National Cancer Institute Egypt

Abstract

<h3>Background</h3> Survival rates of paediatric rhabdomyosarcoma have improved tremendously during the past decade by the development of risk stratification. This approach has favoured tailoring of treatment using multi-therapeutic modalities. <h3>Methods</h3> Patients were given upfront surgical resection followed by systemic chemotherapy using the vincristine/actinomycin-D/cyclophosphamide (VAC) regimen, with subsequent further local control by surgery or radiotherapy, or both, according to risk stratification into low, intermediate, and high risk groups. <h3>Findings</h3> The study included 40 patients; their median age was 3.5years (range: 8months to 17years), 28 were boys and 12 were girls. 2-year overall survival (OS) and event-free survival (EFS) were 87% and 45% respectively. After univariate analysis, OS was 100% for a complete response versus 92% for only partial response (<i>p</i>=0.03). OS was 94% if there were no distant metastasis versus 66% if metastasis was present (<i>p</i>=0.024). On the other hand, EFS was 88% for complete response versus 46% for partial response (<i>p</i><0.001), and was 80% if upfront surgery was done versus 33% if only simple biopsy was taken (<i>p</i>=0.03). Local radiotherapy versus no radiotherapy was highly associated with EFS, with a difference of 75% versus 0%, respectively (<i>p</i><0.001). <h3>Interpretation</h3> Complete response and extent of disease are two important factors influencing survival in childhood rhabdomyosarcoma. Both local control measures, surgical resection and radiation therapy, and treatment response are important variables predicting EFS. The poor outcome of patients with metastatic disease necessitates further therapeutic approaches to improve survival.