Key result
Surgery plus adjuvant chemotherapy yields recurrence-free survival in 3 pediatric extraosseous Ewing sarcoma cases.
Why the study?
Extraosseous Ewing Sarcomas are rare tumors with clinical presentations depending on tumor location, and their characteristics and outcomes in unusual cases need description.
Case Report (n=3)
Surgical excision followed by adjuvant chemotherapy according to the EURO-E.W.I.N.G protocol resulted in survival without recurrence or metastasis at 1 to 4 years in three children with uncommon presentations of extraosseous Ewing sarcoma.
Supports consideration of EURO-E.W.I.N.G protocol in rare pediatric extraosseous Ewing sarcoma; leaves open need for prospective validation.
Ewing sarcoma (ES), described as a diffuse endothelioma of the bone, is divided into two categories: osseous and extraosseous, which mainly affects adolescents. Extraosseous Ewing Sarcomas (EES) are rare tumors originating from soft tissues. Their clinical presentation depends mainly on the primary location of the tumor and are highly chemosensitive and radiosensitive. The purpose of this study was to describe the clinical characteristics and outcomes of 3 children with EES and uncommon presentation treated in our Unit. The diagnosis of EES was confirmed by biopsy and cytogenetic analysis with fluorescence in situ hybridization (FISH). Surgical excision was planned as primary treatment, followed by adjuvant chemotherapy according to EURO-E.W.I.N.G protocol. To date, all patients are alive, 1, 3 and 4 years after completion of treatment, with no signs of recurrence or metastasis.
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Ioannidou et al. (2022) conducted a case report in Extraosseous Ewing Sarcoma (n=3). Surgical excision and adjuvant chemotherapy was evaluated on Survival and recurrence. Surgical excision followed by adjuvant chemotherapy resulted in all 3 children with extraosseous Ewing sarcoma remaining alive with no signs of recurrence or metastasis at 1, 3, and 4 years of follow-up.
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