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Ewing's sarcoma

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 European Reference Network on Genetic Tumour Risk Syndromes (GENTURIS/ERN PaedCan/EURACAN/ESMO 2021) and the National Comprehensive Cancer Network (NCCN 2021).


1.Diagnostic investigations

History and physical examination: as per ESMO 2021 guidelines, elicit medical history and perform physical examination in the initial work-up of patients with suspected primary bone sarcoma.
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  • Imaging for staging

  • Laboratory tests

2.Diagnostic procedures

Biopsy and pathology: as per ESMO 2021 guidelines, perform biopsy in the initial work-op of patients with suspected primary bone sarcoma.
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3.Medical management

Setting of care: 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.

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  • Management of local/locoregional disease

  • Management of advanced/metastatic disease

4.Specific circumstances

Patients with extraskeletal ES
Treat patients with extraskeletal ES following the same principles as for bone ES, incorporating chemotherapy in all patients and radiotherapy in most patients.
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.

5.Patient education

Fertility consultation: consider offering fertility consultation at initial presentation of patients with ES.

6.Follow-up and surveillance

Surveillance: as per ESMO 2021 guidelines, consider obtaining follow-up approximately every 3 months for the first 2 years, every 6 months for years 3-5, every 6-12 months for years 5-10, and every 0.5-1-2 years thereafter.
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  • Management of relapse