Abstract
Primary bone tumours are rare, accounting for <0.2% of malignant tumours registered at the EUROCARE database. They have a relatively high incidence in children and adolescents, but are still numerically outnumbered by benign bone tumours, which clinically may have a similar presentation. They are also frequently difficult to recognize as malignant by clinicians, radiologists as well as pathologists and this leads to major diagnostic difficulties in non-specialized centres. One of the main recommendations of this guideline is that all patients with a suspected primary malignant bone tumour should be referred to a bone sarcoma reference centre or an institution belonging to a specialized bone sarcoma network before biopsy.
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