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Ewing's sarcoma

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of Ewing's sarcoma are prepared by our editorial team based on guidelines from the National Comprehensive Cancer Network (NCCN 2025) and the European Reference Network for Paediatric Cancer (ERN PaedCan/EURACAN/ESMO/ERN GENTURIS 2021).
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2

Diagnostic investigations

History and physical examination: as per NCCN 2025 guidelines, elicit history and perform physical examination in patients presenting with suspected ES.
B
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  • Imaging for staging

  • Laboratory tests

Diagnostic procedures

Biopsy and pathology
As per NCCN 2025 guidelines:
Obtain cytogenetics and/or molecular testing (may require re-biopsy) in patients with ES.
B
Consider performing bone marrow biopsy in patients with ES.
C

Medical management

Setting of care: as per ERN GENTURIS/ERN PaedCan/ESMO/EURACAN 2021 guidelines, manage patients with bone sarcomas at reference centers and/or within reference networks able to provide access to the full spectrum of care and age-specific expertise.
B

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  • Management of local/locoregional disease

  • Management of advanced/metastatic disease

Specific circumstances

Patients with extraskeletal ES
As per ERN GENTURIS/ERN PaedCan/ESMO/EURACAN 2021 guidelines:
Treat patients with extraskeletal ES following the same principles as for bone ES, incorporating chemotherapy in all patients and radiotherapy in most patients.
B
Decide on the number of chemotherapy cycles in patients with cutaneous/subcutaneous ES on an individual case basis, at a multidisciplinary level and with the patient.
B

Patient education

Fertility consultation: as per NCCN 2025 guidelines, consider offering fertility consultation at initial presentation of patients with ES.
C

Follow-up and surveillance

Surveillance: as per NCCN 2025 guidelines, include the following in the surveillance of patients with stable/improved ES following primary treatment:
physical examination
MRI +/- CT (both with contrast of primary site)
chest imaging (X-ray or CT) every 2-3 months
radiographs of primary site
CBC and other laboratory studies as indicated.
B
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  • Management of relapse