title: Extraosseous Ewing Sarcoma: Diagnosis, Prognosis and Optimal Management.
abstract: Extraosseous Ewing sarcomas (EESs) are rare tumours originating from soft tissues. Their clinical picture depends mainly on the primary site of the sarcoma. Patient characteristics and outcomes seem to be different in EES compared to patients with skeletal Ewing sarcoma, with implications for patient care and prognosis. However, multimodality therapeutic strategies are recommended for all types of the Ewing tumour family. The available diagnostic tools include ultrasonographic evaluation and computed tomography (CT) or magnetic resonance imaging as well as histopathologic and immunohistochemical tissue examination. Several histologic and genetic biomarkers have been established, although their utilization needs to be further tested by larger prospective studies. Regarding localized disease, the recommended treatment remains surgery. However, chemotherapy can be added to achieve improved survival, with neoadjuvant regimens showing more promising results than adjuvant regimens. Radiotherapy is an option to obtain local control, although its complications have reduced its utilization. In metastatic or recurrent disease, systematic chemotherapy improves survival.
authors.affiliation: Department of General Surgery, General Hospital of Chalkis, Chalkis, Greece ; 6 Melinas Merkouri Street, Neon Iraklion, 14122 Athens, Greece.
authors.forename: George
authors.initials: G
authors.lastname: Galyfos
authors.affiliation: Department of General Surgery, General Hospital of Chalkis, Chalkis, Greece.
authors.forename: Georgios A
authors.initials: GA
authors.lastname: Karantzikos
authors.affiliation: Department of General Surgery, General Hospital of Chalkis, Chalkis, Greece.
authors.forename: Nikolaos
authors.initials: N
authors.lastname: Kavouras
authors.affiliation: Department of Microbiology, Areteion University Hospital, Athens, Greece.
authors.forename: Argiri
authors.initials: A
authors.lastname: Sianou
authors.affiliation: Department of General Surgery, General Hospital of Chalkis, Chalkis, Greece.
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Palogos
authors.affiliation: 1st Department of Propaedeutic Surgery, University of Athens Medical School, Hippocration Hospital, Athens, Greece.
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Filis
keywords: Biomarkers; Diagnosis; Extraosseous Ewing sarcoma; Soft tissue sarcoma; Therapy