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

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/...

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Detalhes bibliográficos
Autores: Mata Fernández, C.|||0000-0001-9922-0078, Sebio, Ana|||0000-0003-3333-2370, Orcajo Rincón, J., Martín Broto, J., Martín Benlloch, A., Marcilla Plaza, D., López Pousa, Antonio, Gracia, Isidro|||0000-0002-9743-4184, Giuppi, M., Collado Ballesteros, E., Bernabeu, Daniel|||0000-0003-0828-9104, De Álava, Enrique|||0000-0001-8400-046X, Valverde Morales, Claudia Maria
Formato: artículo
Fecha de publicación:2025
País:España
Recursos:Universitat Autònoma de Barcelona
Repositorio:Dipòsit Digital de Documents de la UAB
Idioma:inglés
OAI Identifier:oai:ddd.uab.cat:321765
Acesso em linha:https://ddd.uab.cat/record/321765
https://dx.doi.org/urn:doi:10.1007/s12094-024-03602-5
Access Level:acceso abierto
Palavra-chave:EWSR1
Ewing sarcoma
Malignant bone tumours
Small round cell sarcomas
"Ewing like" sarcoma
Descrição
Resumo: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.

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