“…Therefore, local surgery is an appropriate treatment for patients with metastatic ES. Raciborska et al [25] reported that treatment of isolated lung metastases may have a role in improving prognosis in patients with ES. Letourneau et al [26] reported that resection of pulmonary metastases in pediatric patients with ES improved survival.…”
Background
Metastatic Ewing’s sarcoma (ES) of bone has a poor prognosis. Because there have been few previous studies on the prognostic factors and clinical outcome in patients with ES who have metastases at presentation, the aim of this study was to use the Surveillance, Epidemiology, and End Results (SEER) database to compare the clinical outcome following single and combined radiation treatment and surgery.
Material/Methods
The SEER database was used to identify patients with ES who presented with bone involvement and metastasis between 1973 to 2015. Prognostic analysis was performed using the Kaplan-Meier method and the Cox proportional hazards regression model.
Results
There were 643 patients identified from the SEER database. The 5-year overall survival (OS) and cancer-specific survival (CSS) rates were 33.1% and 34.3%, respectively and the median OS and CSS were 29.0±1.9 and 29.0±2.1 months, respectively. Multivariate analysis identified age <20 years and surgical resection of the primary tumor to be significantly associated with improved OS. Radiation therapy was not an independent predictor of OS or CSS. Radiation therapy alone resulted in a significantly reduced the OS and CSS compared with surgical resection alone. Combined surgery and radiation therapy of the primary tumor did not significantly improve the OS or CSS of patients with ES and metastatic disease when compared with surgery alone.
Conclusions
Age <20 years and surgical resection of the primary tumor were significantly associated with improved OS in patients with primary ES of bone who presented with metastasis.
“…Therefore, local surgery is an appropriate treatment for patients with metastatic ES. Raciborska et al [25] reported that treatment of isolated lung metastases may have a role in improving prognosis in patients with ES. Letourneau et al [26] reported that resection of pulmonary metastases in pediatric patients with ES improved survival.…”
Background
Metastatic Ewing’s sarcoma (ES) of bone has a poor prognosis. Because there have been few previous studies on the prognostic factors and clinical outcome in patients with ES who have metastases at presentation, the aim of this study was to use the Surveillance, Epidemiology, and End Results (SEER) database to compare the clinical outcome following single and combined radiation treatment and surgery.
Material/Methods
The SEER database was used to identify patients with ES who presented with bone involvement and metastasis between 1973 to 2015. Prognostic analysis was performed using the Kaplan-Meier method and the Cox proportional hazards regression model.
Results
There were 643 patients identified from the SEER database. The 5-year overall survival (OS) and cancer-specific survival (CSS) rates were 33.1% and 34.3%, respectively and the median OS and CSS were 29.0±1.9 and 29.0±2.1 months, respectively. Multivariate analysis identified age <20 years and surgical resection of the primary tumor to be significantly associated with improved OS. Radiation therapy was not an independent predictor of OS or CSS. Radiation therapy alone resulted in a significantly reduced the OS and CSS compared with surgical resection alone. Combined surgery and radiation therapy of the primary tumor did not significantly improve the OS or CSS of patients with ES and metastatic disease when compared with surgery alone.
Conclusions
Age <20 years and surgical resection of the primary tumor were significantly associated with improved OS in patients with primary ES of bone who presented with metastasis.
“…Metastasectomy performed at that time showed that all Group 3 and 63% of Group 2 patients had viable disease, but none of the Group 1 patients had viable disease. Patients with a radiographic response to initial chemotherapy had an improved event-free survival, but no effect of metastasectomy was observed 85 . Given the multitude of conflicting reports hampered by poorly controlled data, there is no reliable evidence that metastasectomy in Ewing sarcoma is of therapeutic benefit.…”
Most children who succumb to solid malignancies do so because of the burden of metastatic disease or due to complications associated with the therapy administered to treat metastatic disease. Approximately one-quarter of children with solid tumors will present with metastatic disease, and an additional 20% ultimately develop metastatic disease, most commonly in the lung. The role of surgery in the treatment of metastatic solid tumors, given its disseminated nature, is not intuitive, yet there are circumstances in which surgical resection of metastatic disease can potentially be curative. However, the utility of surgery is very much dependent on histology, and generally is most appropriate for those malignancies with histologies that are refractory to other adjuvant therapies.
“…Treatment of metastatic sites of disease with radiation and sometimes even surgery has been considered as part of consolidation. For example, for patients with pulmonary metastases, non-randomized studies supported the delivery of whole lung irradiation after completion of chemotherapy; (41) and for patients with residual lung metastases after completion of chemotherapy, further surgical resection has been advocated (42). Consideration has also been given to irradiating bone and soft tissue metastases, especially in the setting of oligometastatic disease.…”
Ewing sarcoma (ES) family of tumors includes bone and soft tissue tumors that are often characterized by a specific translocation between chromosome 11 and 22, resulting in the EWS-FLI1 fusion gene. With the advent of multi-modality treatment including cytotoxic chemotherapy, surgery, and radiation therapy, the prognosis for patients with ES has substantially improved. However, a therapeutic plateau is now reached for both localized and metastatic disease over the last two decades. Burdened by the toxicity limits associated with the current frontline systemic therapy, there is an urgent need for novel targeted therapeutic strategies. In this review, we discuss the current treatment paradigm of ES, and explore preclinical evidence and emerging treatments directed at tumor signaling pathways and immune targets.
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