Benign bone tumours are common, usually asymptomatic, and discovered incidentally. Malignant primary bone tumours are uncommon but cause significant morbidity and mortality, particularly in adolescents and young adults. Bony metastases are the tumours most frequently seen in bone. Malignant bone tumours typically present with localized pain or swelling. With patients in whom the diagnosis is not clearly metastatic disease, determination of tumour size and extent is best achieved by magnetic resonance imaging, and bone biopsy is mandatory to establish a precise histological diagnosis. Osteosarcoma, chondrosarcoma, and Ewing sarcoma are the three commonest primary bone tumours. In determining management, the main clinical distinction is between localized and metastatic disease. Non-metastatic primary tumours are treated with surgery (when possible) and chemotherapy (osteosarcoma and Ewing sarcoma, sometimes chondrosarcoma). Symptomatic bony metastases are usually treated with external beam radiotherapy.
Ubiquitin-specific proteases as therapeutic targets in paediatric primary bone tumours?
