NICE guideline [NG12] Suspected cancer: recognition and referral 1.11 Sarcomas. Last updated: Apr 2026.
Article Last Updated:04/07/2026
Bone Tumours
Bone tumours may be primary or secondary. Primary bone tumours arise from bone or cartilage, and include benign lesions as well as malignant bone sarcomas such as osteosarcoma, Ewing sarcoma and chondrosarcoma. Secondary bone tumours, or bone metastases, are more common and occur when cancer spreads to bone from another primary site.
Primary Bone Tumours
Primary Malignant Bone Tumours
There are 3 main types of malignant primary tumours, which are all sarcomas: [Ref]
Chromosomal translocation between chromosome 11 and 22
EWSR1 gene on chromosome 22 fuses to the FLI1 gene on chromosome 11
Pre-existing primary benign bone tumours (e.g. osteochondromas, enchondromas)
Previous radiation therapy
Symptoms
Bone pain (esp. bone pain at night is a red flag)
Loss of function of the affected limb (e.g. limping)
Unexplained lump / swelling
Progressive lesion / symptoms
Possible pathological fractures
Diagnostic / referral pathway
Referral pathway / indications (NICE):
Children and young people:
Clinically suspected bone sarcoma → very urgent X-ray (within 48 hours)
If X-ray suggests possible bone sarcoma → very urgent referral to specialist (within 48 hours)
Adults:
Consider a suspected cancer pathway referral if X-ray suggests possible bone sarcoma
Standard investigations:
1st line: X-ray
Confirmatory test: MRI-guided core needle biopsy
Additional investigations:
Staging with whole-skeleton (whole body CT/MRI/PET-CT or isotope bone scan) and chest imaging (CT chest)
Additional investigations:
Staging with whole-skeleton imaging (whole body CT/MRI/PET-CT or isotope bone scan)
Molecular genetic testing to confirm EWSR1 gene rearrangement
MDT involvement is necessary
Most commonly affected locations
Usually arise in the metaphysis of the bone
Most commonly develop around the knee joint
Predominantly in adults, the axial skeleton, pelvis or craniofacial bones can also be involved
Usually arise in the diaphysis of the bone
Most commonly develop in long bones (e.g. femur, humerus), pelvis, vertebral column, ribs
Most commonly develop in long bones
Other locations: pelvis, ribs, scapula
Radiographic features
Aggressive lesion with mixed lytic / sclerotic changes
Sunburst appearance
Codman triangle
“Moth-eaten” lytic lesions
Laminated onion-skin periosteal reaction
Possible Codman triangle
Lytic expansile lesion
Rings-and-arcs calcification
Popcorn-like calcification
Management
Standard curative approach:
Neoadjuvant (pre-operative) chemotherapy
Surgical resection
Adjuvant (post-operative) chemotherapy
Standard 1st line chemotherapy: MAP (methotrexate + doxorubicin + cisplatin)
Inoperable disease: consider radiotherapy
Standard curative approach:
Intensive chemotherapy (VDC/IE) – to be used as neoadjuvant induction and consolidation therapy
Surgical resection
Inoperable disease: definitive radiotherapy
Standard curative approach:
Surgical resection
Chondrosarcomas do NOT respond well to chemotherapy and radiotherapy
Inoperable disease: consider radiotherapy
Primary Benign Bone Tumours
Disclaimer:
Primary benign bone tumours are generally less high-yield than primary malignant bone tumours for exam purposes, as they are usually less aggressive and are not typically life-threatening.
Treatment of choice for localised moderate to severe bone pain: external beam radiotherapy
If the pain is poorly localised / pain recurs in previously irradiated sites → systemic therapy
Bisphosphonates
Denosumab (RANKL inhibitor)
Hypercalcaemia of malignancy
Immediate management: IV fluid rehydration
Further therapy:
1st line: IV bisphosphonates (e.g. zoledronic acid, pamidronate disodium)
Consider denosumab if refractory to bisphosphonates or if bisphosphonates are not appropriate
Metastatic spinal cord compression
Oral dexamethasone (if suspected clinically, give the steroid immediately without waiting for MRI confirmation)
Confirm the diagnosis with MRI
Definitive management: surgical decompression
If patient is not suitable for surgery → radiotherapy
Pathological and impending fractures
Pathological fracture → orthopaedic stabilisation (e.g. internal fixation with intramedullary nail or long-term prosthesis) followed by post-operative fractionated radiotherapy
Impending fractures → prophylactic surgical stabilisation is generally preferred over waiting to fix a fracture after it occurs
Prevention of skeletal-related events
Once the diagnosis of bone metastasis is made, start one of the following:
Denosumab (RANKL inhibitor) – generally preferred for its higher efficacy, better renal safety, and convenience, or
Bisphosphonates
Patient must be on calcium and vitamin D supplementation to avoid hypocalcaemia, and undergo a dental evaluation before initiation to reduce risk of ONJ.