Consultant orthopaedic surgeon Amit Kumar takes a look at the Sarcoma UK guidelines
Soft tissue tumours (sarcomas) are very rare cancers that are usually found on the arms, legs and trunk. They can however affect any part of the body, and account for less than 1 per cent of all cancers in the UK. Bone sarcomas, similarly rare, present in any bone and may present with incidental radiographic changes or bone/joint pain.
Sarcoma UK published recently that almost 5,300 new cases are diagnosed per year in the UK. Being a rare cancer, early diagnosis and management is imperative and patients should be referred to one of the specialised sarcoma centres in the UK.
It’s rare for a GP to see in their career, however when they do an early referral should be made to improve patient’s outcomes. Orthopaedic surgeons are likely to be referred a patient with an unexplained lump or limb/joint pain that requires further assessment. Subsequently you need to know what investigations to request and where to refer.
Recent data from Sarcoma UK has shown that the majority of clinicians might ignore early symptoms and only one in four doctors might be able to diagnose these signs. Sarcomas are common with increasing age, but can occur in any age group. The survival rate at five years is approximately 51 per cent and this hasn’t improved over the past decade.
Symptoms and referral criteria of soft tissue sarcoma include an unexplained lump, which is increasing in size, larger than 5cm (a golf ball), painful (majority are not) and deep within the tissues, but not always. Sometimes lumps which are small in size can present as an aggressive lesion. There may be evidence of skin changes, discolouration, and fungating lesions, particularly if there’s a history of previous surgery or radiotherapy. Consider other primary cancers that can metastasise.
Table 1: Referral criteria
| Lumps > 5 cm |
| Lump increasing in size |
| Lump deep to fascia |
| Pain |
| Radiographic bone changes |
| Previously excised lump (recurrence) |
| Previous radiotherapy |
Bone sarcomas are rarer than their soft tissue counterpart, but can have devastating consequences if early symptoms and signs are not recognised and investigated. Joint and limb pain may be the only presentation, but also look for swelling and unexplained fractures. An unexplained limp with mobility issues may also be present. Bone changes are usually seen by incidental X-rays taken secondary to trauma to the affected site. Osteosarcoma, Ewing sarcoma and Chordoma are the commonest types.
If you suspect a sarcoma, obtain a plain film X-ray and MRI scan with contrast at the first instance and consider an early suspected cancer referral to your local sarcoma unit. Investigations should not delay your referral however.
In England and Wales, there are 15 sarcoma specialist centres.
Any patient with a suspected soft tissue or bone sarcoma should be referred to your diagnostic centres for clinical assessment, diagnostics and management. Scotland and Northern Ireland also have sarcoma centres managing suspected sarcoma cases. All cases are discussed in a weekly multidisciplinary team. The core members of the MDT include sarcoma surgeons, radiologists with specialist interest in sarcoma, sarcoma histopathologists, clinical nurse specialists, clinical and medical oncologists and an MDT coordinator.
With more than 100 subtypes, recognition of the symptoms is crucial to ensure prompt management. Assessment is best done by a specialised sarcoma surgeon with a thorough history and examination. All lumps should be imaged appropriately (ultrasound or MRI scan) and, if possible, a biopsy may be taken for review by a sarcoma histopathologist. There is no role for fine needle aspiration, core biopsies must be taken and best done by the sarcoma team.
Management includes surgical removal of the sarcoma (i.e. wide local excision or radical) along with radiotherapy or chemotherapy, depending on sarcoma type. Reconstructive methods may be used, especially if a wide segment of bone is removed to permit mobility and function, such as an endoprosthetic replacement. There also may be clinical trials patients may be offered in addition to genomic sequencing.
Pitfalls in managing patients suspected with sarcoma include lengthy delays (both in primary and secondary care) and numerous consultations before a diagnosis is found. This may be due to a diagnosis of sarcoma not being considered, community scans being performed which are difficult to interpret or access, and not knowing who the right clinician the patient should be referred to.
Patients with a persisting haematoma should be suspected of having a sarcoma if after repeat imaging the lesion remains.
Pathological bone fractures and haematoma not resolving should also raise concern if the presenting history and mechanism doesn’t fit the clinical injury. Young patients with low energy trauma, particularly of the long bones, should be investigated prior to surgery.
It is important to rule out a primary bone sarcoma or metastatic bone disease from a primary lesion elsewhere. It is also important not to rush into surgery until the orthopaedic surgeon is sure it is not a cancerous lesion causing the fracture.
Sarcoma specification
The sarcoma service specification has recently been revised after public consultation and provides guidance for patients and clinicians managing sarcoma cases. The notable updates were:
Requirement that sarcoma networks develop network-wide treatment protocols and pathways; particularly improving relationships with primary care and other referrers.
All sarcoma cases must be referred to the specialist sarcoma MDT.
Improvement and clarity on the working relationship between sarcoma networks and children and teenage and young adult networks.
For information and useful resources for information and patient support, search for Sarcoma UK and Bone Cancer Research Trust.
Author
Amit Kumar is a consultant orthopaedic and oncological surgeon at Greater Manchester & Oswestry Sarcoma Service. He is also the SAG chair for Greater Manchester and Clinical Director for Sarcoma Greater Manchester Cancer.
