Table of contents
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 for Paediatric Cancer (ERN PaedCan/EURACAN/ESMO/ERN GENTURIS 2021), the American College of Radiology (ACR 2020), and the Endocrine Society (ES 2014).
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Diagnostic investigations
Initial evaluation
As per ERN GENTURIS/ERN PaedCan/ESMO/EURACAN 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 ERN GENTURIS/ERN PaedCan/ESMO/EURACAN 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 ERN GENTURIS/ERN PaedCan/ESMO/EURACAN 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 craniofacial osteosarcoma
As per ERN GENTURIS/ERN PaedCan/ESMO/EURACAN 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