Bone sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

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. Primary bone tumours are considerably outnumbered by metastases to the bone in older patients, which in some instances might mimic the presentation of a primary bone tumour. The presence of non-mechanical pain or night pain around the knee of a person in this or indeed any age group should cause concern and lead to further immediate investigation. Swelling will only be present if the tumour has progressed through the cortex and distended the periosteum.