Soft tissue sarcoma
|AYAs presenting with symptoms suggestive of soft tissue sarcoma should be referred to a sarcoma specialist affiliated with a Youth Cancer Service where available.||D|
An urgent specialist referral should be made in AYAs presenting with an unexplained mass (at almost any site) that has one or more of the following features:
Symptoms and signs
Sarcomas often occur on the limbs and trunk, but can occur anywhere that connective tissue is present. The most common symptom is an unexplained, painless, progressively enlarging mass in the soft tissue; signs include a palpable mass, proptosis and cranial nerve palsy or nasal obstruction, depending on the location. Other symptoms may be present if the tumour interferes with particular body functions.
A soft tissue mass in an unusual location may give rise to misleading local and persistent unexplained symptoms and signs in adolescents and young adults (AYAs), and the possibility of sarcoma should be considered by the general practitioner (GP).
Where an unexplained mass (at almost any site) is deep to the fascia, non-tender, progressively enlarging or associated with a regional lymph node that is enlarging greater than 2 cm in diameter, an urgent specialist referral (to a sarcoma specialist affiliated with a Youth Cancer Service where available) should be made.
Appropriate imaging investigation of the lump (ultrasound, MRI or CT scan) should be organised by the GP, but this should not delay referral to a specialist. The patient should be referred to a specialist before any biopsy or fine needle aspirate and surgical management should occur at a regional sarcoma service. Biopsies in primary care should be avoided.
Although most soft tissue sarcomas develop sporadically, a number of genetic disorders including neurofibromatosis, retinoblastoma, Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, Recklinghausen’s disease and FAP have been associated with an increased risk. Prior radiotherapy may also cause soft tissue sarcomas.
- National Collaborating Centre for Cancer. Improving Outcomes for People with Sarcoma: The Manual. London: National Institute for Health and Clinical Excellence; 2006.
- Bleyer A. CAUTION! Consider cancer: common symptoms and signs for early detection of cancer in young adults. Semin Oncol 2009 Jun;36(3):207-12 Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/19460578.
- 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.
- New Zealand Guidelines Group. Suspected cancer in primary care: guidelines for investigation, referral and reducing ethnic disparities. Wellington: New Zealand Guidelines Group; 2009.