Sistema de Salut de Catalunya
Institut Català de la Salut
[Mata Fernández C] Pediatric and Adolescent Oncohaematology Unit, Hospital Materno-Infantil Gregorio, Marañón Madrid, Spain. [Sebio A] Medical Oncology Department, Hospital Sant Pau, Barcelona, Spain. [Orcajo Rincón J] Nuclear Medicine Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain. [Martín Broto J] Medical Oncology Department, Fundación Jimenez Diaz University Hospital, University Hospital General de Villalba, and Instituto de Investigacion Sanitaria Fundacion Jimenez Diaz (IIS/FJD; UAM), Madrid, Spain. [Martín Benlloch A] Section Spine Unit, Orthopaedic and Traumatology Department, Dr. Peset University Hospital, Valencia, Spain. [Marcilla Plaza D] Department of Pathology, Hospital Universitario Virgen del Rocío, Sevilla, Spain. [Valverde Morales C] Servei d’Oncologia Mèdica, Vall d’Hebron Hospital Universitari, Barcelona, Spain
Vall d'Hebron Barcelona Hospital Campus
2025-04-01T11:33:28Z
2025-04-01T11:33:28Z
2025-03
Ewing sarcoma; Malignant bone tumours; Small round cell sarcomas
Sarcoma d'Ewing; Tumors ossis malignes; Sarcomes de cèl·lules rodones petites
Sarcoma de Ewing; Tumores óseos malignos; Sarcomas de células redondas pequeñas
Ewing sarcoma is a small round-cell sarcoma characterized by gene fusion involving EWSR1 (or another TET family protein like FUS) and an ETS family transcription factor. The estimated incidence of this rare bone tumor, which occurs most frequently in adolescents and young adults, is 0.3 per 100,000/year. Although only 25% of patients with Ewing sarcoma are diagnosed with metastatic disease, historical series show that this is a systemic disease. Patient management requires multimodal therapies—including intensive chemotherapy—in addition to local treatments (surgery and/or radiotherapy). In the recurrent/refractory disease setting, different approaches involving systemic treatments and local therapies are also recommended as well as patient inclusion in clinical trials whenever possible. Because of the complexity of Ewing sarcoma diagnosis and treatment, it should be carried out in specialized centers and treatment plans should be designed upfront by a multidisciplinary tumor board. These guidelines provide recommendations for diagnosis, staging, and multimodal treatment of Ewing sarcoma.
Article
Published version
English
Sarcoma d'Ewing - Tractament; Sarcoma d'Ewing - Diagnòstic; Ossos - Càncer - Tractament; Ossos - Càncer - Diagnòstic; ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Therapeutics::Combined Modality Therapy; DISEASES::Neoplasms::Neoplasms by Site::Bone Neoplasms; DISEASES::Neoplasms::Neoplasms by Histologic Type::Neoplasms, Connective and Soft Tissue::Neoplasms, Connective Tissue::Neoplasms, Bone Tissue::Osteosarcoma::Sarcoma, Ewing; Other subheadings::Other subheadings::/therapy; DISEASES::Neoplasms::Neoplasms by Histologic Type::Neoplasms, Connective and Soft Tissue::Neoplasms, Connective Tissue::Neoplasms, Bone Tissue::Osteosarcoma::Sarcoma, Ewing; Other subheadings::Other subheadings::/diagnosis; TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::terapéutica::tratamiento combinado; ENFERMEDADES::neoplasias::neoplasias por localización::neoplasias óseas; ENFERMEDADES::neoplasias::neoplasias por tipo histológico::neoplasias de tejido conjuntivo y de tejidos blandos::neoplasias de tejido conjuntivo::neoplasias de tejido óseo::osteosarcoma::sarcoma de Ewing; Otros calificadores::Otros calificadores::/terapia; ENFERMEDADES::neoplasias::neoplasias por tipo histológico::neoplasias de tejido conjuntivo y de tejidos blandos::neoplasias de tejido conjuntivo::neoplasias de tejido óseo::osteosarcoma::sarcoma de Ewing; Otros calificadores::Otros calificadores::/diagnóstico
Springer
Clinical and Translational Oncology;27
https://doi.org/10.1007/s12094-024-03602-5
Attribution 4.0 International
http://creativecommons.org/licenses/by/4.0/
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