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

Cristina Mata, Ana Sebio, Javier Orcajo Rincón, Javier Martín Broto, Juan Antonio Martín Benlloch, David Marcilla Plaza, Antonio López Pousa, I. Gracia Alegría, Martina Giuppi, Erika Collado Ballesteros, Daniel Bernabeu Taboada, Enrique de Alava, Claudia M. Valverde

• 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.