|AYAs presenting with symptoms suggestive of bone cancer should be referred to a sarcoma specialist affiliated with a Youth Cancer Service where available.||D|
|AYAs with increasing, unexplained or persistent bone pain, tenderness or swelling, particularly pain at rest (and especially if not in the joint), persistent back pain or an unexplained limp should be investigated urgently by the GP including x-ray, and referred to a specialist if considered concerning.||C|
|GPs should consider the possibility of a bone sarcoma in AYAs irrespective of history of an injury.||C|
|GPs should consider primary or secondary bone cancer in AYAs with suspected spontaneous fracture and refer patients for an immediate x-ray (completed and reported within 5 days).||C|
|If an x-ray indicates that bone cancer is a possibility, an urgent specialist referral should be made.||C|
|If the x-ray is normal but the patient remains symptomatic, the x-ray should be repeated and the patient referred to a specialist. Other investigations may be ordered as appropriate.||C|
Symptoms and signs
Symptoms of bone tumours (including Osteosarcoma and Ewing Sarcoma) vary depending on the part of the body affected and the size of the tumour. Limbs are the most common site for bone tumours, especially around the knee in the case of osteosarcoma.
Most patients do not present to primary care with a palpable abnormality. Regional bone pain, often related to strain, is the most common symptom reported. However, bone tenderness, limping, swelling, non-resolution of prior athletic injury or other physical trauma may also be present. General practitioners (GPs) should consider primary or secondary bone cancer in adolescents and young adults (AYAs) with suspected spontaneous fracture; and bone sarcoma irrespective of history of an injury.
As the presence of a palpable mass is an important clinical feature of bone cancer, thorough physical examination is recommended.
If primary bone tumour or sarcoma is suspected (especially if symptoms are increasing, unexplained or persistent) the first investigation should be an x-ray. Other imaging such as MRI, CT scans or bone scans are not as useful in evaluating bone lesions.
If an x-ray indicates bone cancer, urgent specialist referral (to a sarcoma specialist affiliated with a Youth Cancer Service where available) should be made. Surgical management of AYAs with bone sarcoma should occur at a regional sarcoma service.
If the x-ray is normal but the patient remains symptomatic, the x-ray should be repeated and the patient referred to a specialist. Blood tests may include FBC, blood film, PTH, LFTs, LDH, calcium (plasma or serum), phosphate (plasma or serum), albumin (plasma or serum), protein (total), protein electrophoresis (serum), CRP and Bence Jones protein (urine). Other investigations such as FNAB, tissue biopsy, bone biopsy (fracture site), bone marrow aspiration and trephine biopsy may be considered as appropriate.
Osteosarcoma has been associated with radiotherapy treatment for cancer during childhood (risk increases with treatment at a younger age and/or being treated with higher doses of radiation), chemotherapy treatment for cancer during childhood (e.g. alkylating agents) and genetic disorders such as retinoblastoma, Li-Fraumeni syndrome and Rothmund-Thomson syndrome. Rapid bone growth in utero (i.e. high-birth weight) and at puberty (i.e. above average height) may also increase the risk of osteosarcoma.
However, bone tumours can occur in AYAs without any of these risk factors.
- Adolescent and Young Adult Working Party of the Statewide Cancer Clinical Network. South Australian Adolescent and Young Adult Cancer Care Pathway: Optimising outcomes for all adolescent and young adult South Australians with a cancer diagnosis. Adelaide: South Australia Department of Health; 2010.
- National Collaborating Centre for Primary Care. Referral Guidelines for Suspected Cancer. Clinical guideline 27. London: National Institute for Health and Clinical Excellence; 2005.
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- New Zealand Guidelines Group. Suspected cancer in primary care: guidelines for investigation, referral and reducing ethnic disparities. Wellington: New Zealand Guidelines Group; 2009.
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- National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines™) Bone Cancer Version 1.2012. 2011 Abstract available at http://www.nccn.org/professionals/physician_gls/pdf/bone.pdf.
- Royal College of Pathologists of Australia. RCPA Manual. Sydney: Royal College of Pathologists of Australia; 2011.
- Mirabello L, Pfeiffer R, Murphy G, Daw NC, Patiño-Garcia A, Troisi RJ, et al. Height at diagnosis and birth-weight as risk factors for osteosarcoma. Cancer Causes Control 2011 Jun;22(6):899-908 Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/21465145.