Sarcoma de Ewing
ICD-10 C40 · ICD-11 2B52

Tratamiento de primera línea del sarcoma de Ewing: quimioterapia de intervalo comprimido y control local

Escenario clínico

El sarcoma de Ewing es un tumor óseo primario agresivo que requiere tratamiento combinado sistémico y local desde el inicio. El tratamiento de primera línea se inicia de forma inmediata, y la respuesta a la quimioterapia orienta las decisiones de tratamiento local del tumor primario.

Enfoque terapéutico

La estrategia preferida de primera línea implica quimioterapia combinada de intervalo comprimido, integrada con el tratamiento local definitivo del tumor primario —resección quirúrgica y/o radioterapia— según las características del tumor, la localización anatómica y el estado general del paciente.

Los detalles completos del régimen, el calendario de fármacos, el número de ciclos y los criterios de decisión del tratamiento local se encuentran en el protocolo estructurado a continuación.

Objetivos clave del tratamiento
Acceso inmediato a regímenes estructurados basados en evidencia

References

DOI: 10.1038/s41416-024-02868-4

Greater treatment intensity is linked to better outcomes: a two-weekly interval-compressed VCD/IE induction was demonstrated to be more effective than the same regimen given three-weekly and VDC/IE induction followed by IE/VC consolidation has better outcomes than VIDE induction and VAI or VAC consolidation and is now the preferred first-line treatment for all patients who are medically fit to receive it.

Complete removal of the primary tumour (meaning the parts of all anatomical structures involved in the original tumour volume) provides optimal local control but is not always feasible, for example, because critical anatomical structures are involved.

Radiotherapy should be considered in addition to surgery if there is a poor radiological or histological response to chemotherapy, the surgical margins are inadequate, the tumour is large or is in a high-risk area (e.g. pelvis).

Definitive radiotherapy is frequently recommended for tumours judged to be inoperable, those in anatomic locations where complete removal would cause unacceptable morbidity (e.g. pelvic and sacral tumours), in patients at unacceptable risk of significant surgical complications, and if the prognosis is poor (e.g. widespread bone metastases) such that morbidity of surgery is not appropriate.

If there is a poor histological response (≤90% necrosis) to pre-operative chemotherapy, even if the resection margins are negative.

Tumour volume change can be seen on MRI, and reliably reflects chemotherapy response particularly if late.

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