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Osteosarcoma

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of osteosarcoma 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), the American College of Radiology (ACR 2020), and the Endocrine Society (ES 2014)....
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Diagnostic investigations

Initial evaluation
As per GENTURIS/ERN PaedCan/EURACAN/ESMO 2021 guidelines:
Evaluate patients with suspected primary bone sarcoma at a sarcoma reference center with history, physical examination, imaging, and biopsy.
B
Obtain baseline measurements of serum ALP and LDH levels.
B
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  • Diagnostic imaging

  • Imaging for staging

Diagnostic procedures

Biopsy and histopathology
As per GENTURIS/ERN PaedCan/EURACAN/ESMO 2021 guidelines:
Use the 2020 WHO classification for pathological diagnosis supported by ancillary testing whenever relevant.
B
Describe surgical specimens, tumor size and local extent of spread, site, status of surgical margins, and percentage of pathological response to preoperative chemotherapy.
B

Medical management

Management of low-grade disease: as per GENTURIS/ERN PaedCan/EURACAN/ESMO 2021 guidelines, offer surgery alone in patients with low-grade central and parosteal osteosarcoma, as they have a low metastatic potential.
B
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  • Management of high-grade disease (surgery)

  • Management of high-grade disease (chemotherapy)

  • Management of high-grade disease (radiotherapy)

  • Management of metastatic disease

  • Management of relapse

Specific circumstances

Patients with carniofacial osteosarcoma
As per GENTURIS/ERN PaedCan/EURACAN/ESMO 2021 guidelines:
Treat high-grade craniofacial osteosarcoma similarly to high-grade osteosarcoma at other sites.
B
Consider offering radiotherapy when complete surgery is not feasible and in patients undergoing resection with positive margins.
C

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  • Patients with Paget's disease

Follow-up and surveillance

Surveillance: as per GENTURIS/ERN PaedCan/EURACAN/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.
C
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