Clinical practice guidelines for the treatment of Ewing sarcoma (Spanish Sarcoma Research Group‑GEIS)

• Cristina Mata Fernández ^[4] ; Ana Sebio ^[5] ; Javier Orcajo Rincón ^[1] ; Javier Martín Broto ^[6] ; Antonio Martín Benlloch ^[7] ; David Marcilla Plaza ^[2] ; Antonio López Pousa ^[5] ; Isidro Gracia Alegría ^[8] ; Martina Giuppi ^[9] ; Erica Collado Ballesteros ^[10] ; Daniel Bernabeu ^[11] ; Enrique de Alava ^[3] ; Claudia Valverde Morales ^[12]
  1. [1] Hospital General Universitario Gregorio Marañón

     Hospital General Universitario Gregorio Marañón

     Madrid, España

     
  2. [2] Hospital Universitario Virgen del Rocío

     Hospital Universitario Virgen del Rocío

     Sevilla, España

     
  3. [3] Universidad de Sevilla

     Universidad de Sevilla

     Sevilla, España

     
  4. [4] Pediatric and Adolescent Oncohaematology Unit, Hospital Materno-Infantil Gregorio, Marañón Madrid, Spain
  5. [5] Medical Oncology Department, Hospital Sant Pau, Barcelona, Spain
  6. [6] 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
  7. [7] Section Spine Unit. Orthopaedic and Traumatology Department, Dr. Peset University Hospital, Valencia, Spain
  8. [8] Orthopaedic Oncology Unit, Orthoapedic and Traumatology Department, Hospital Sant Pau, Barcelona, Spain
  9. [9] Sarcoma patient, Madrid, Spain
  10. [10] Radiation Oncologist, Hospital Universitari i Politécnic La Fe , Valencia, Spain
  11. [11] Chief of Musculo-skeletal Radiology Section, Radiodiagnosis Service Hospital General Universitario La Paz, Madrid, Spain
  12. [12] Medical Oncology Department, Hospital Universitari Vall d´Hebrón, Barcelona, Spain

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• Localización: Clinical & translational oncology, ISSN 1699-048X, Vol. 27, Nº. 3, 2025, págs. 824-836
• Idioma: inglés
• Texto completo no disponible (Saber más ...)
• Resumen

  • 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, diferent 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.