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

Tratamento de primeira linha do sarcoma de Ewing: quimioterapia com intervalo comprimido e controle local

Cenário Clínico

O sarcoma de Ewing é um tumor ósseo primário agressivo que requer tratamento sistêmico e local combinado desde o início. O manejo de primeira linha é iniciado prontamente, com a resposta à quimioterapia orientando as decisões de tratamento local para o tumor primário.

Abordagem Terapêutica

A estratégia de primeira linha preferida envolve quimioterapia combinada com intervalo comprimido, integrada ao tratamento local definitivo do tumor primário — ressecção cirúrgica e/ou radioterapia — dependendo das características do tumor, localização anatômica e condição clínica do paciente.

Os detalhes completos do regime, cronograma de medicamentos, contagem de ciclos e critérios de decisão para o tratamento local estão no protocolo estruturado abaixo.

Principais Objetivos do Tratamento
Acesso Imediato a Regimes Estruturados Baseados em Evidências

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.

View source ↗