Survival Outcome of Rhabdomyosarcoma (RMS) in Egyptian Children: Experience of the National Cancer Institute-Egypt

摘要

Background. Survival rates of pediatric Rhabdomyosarcoma (RMS) have been tremendously improved during the last decade by the development of risk stratification. This has favored tailoring treatment using multi-therapeutic modalities. Methods. Upfront surgical resection was followed by systemic chemotherapy using Vincristine/Actinomycine-D/Cyclophosphamide (VAC) regimen with subsequent further local control by surgery and or radiotherapy according to risk stratification status. Results. Study included 40 patients; their median age was 3.5 years (range: 8 m to 17 yrs) with M/F: 28/12. The 2 years Overall Survival (OS) and Event Free Survival (EFS) for all study patients was 87% and 45% respectively. By univariate analysis, OS was 100% if CR (complete response) versus 92% if PR (partial response) (p=0.03), and was 94% if no distant metastasis versus 66% if present (p=0.024). On the other hand, EFS was 88% with CR versus 46% in PR patients (p <0.001), and was 80% if upfront surgery was done versus 33% if only simple biopsy taken (p=0.03). Local radiotherapy versus no radiotherapy was highly associated with EFS difference as well (75% versus 0%, respectively; p<0.001). In multivariate analysis, local radiotherapy found to be an independent prognostic factor of EFS (95% CI: 2.5-31). Conclusion. Disease extent as well as treatment response are two important factors influenced survival in our RMS patients. Local control measures including surgical resection as well as radiotherapy are crucial variables that predicted EFS. The poor outcome of patients with metastatic disease necessitates further therapeutic approaches.