Sir Stanford Cade was one of the pioneers of radiotherapy and worked at the Westminster Hospital between 1924 and 1960. Amongst his extraordinary output he published a thoughtful paper on the treatment of osteogenic sarcoma in the prechemotherapy era (1). He reasoned that early amputation had no impact on survival in osteosarcoma, and therefore that early metastasis was the norm. Furthermore he argued that to amputate the limbs of patients with such a poor prognosis was unreasonable and mutilating. The Cade regime therefore involved high dose radiotherapy to the primary tumour site, followed by secondary amputation if the patient survived long enough. He was able to demonstrate clinical, radiological and pathological responses to radiotherapy in this relatively radioresistant tumour. His description defined treatment for osteosarcoma in the prechemotherapy era.
Journal of the Royal College of Surgeons of Edinburgh. 1955 Dec ;1(2):79–111.
The principle of multidisciplinary team working has been enshrined in NHS culture since the publication of the NHS Cancer Plan in 2000. It is particularly important that sarcomas are managed by specialist multidisciplinary teams because many aspects of diagnosis require discussion between clinicians, radiologists and pathologists, and the multi-modality nature of treatment for many sarcomas means that coordination is needed in order to tailor treatment to the needs of the individual patient. Sarcoma specialist multidisciplinary teams should include specialists in:
- orthopaedic, plastic, general and thoracic surgery
- medical (and paediatric) oncology
- clinical (radiation) oncology
NICE guidance suggests the following:
1.13 Bone cancer and sarcoma
1.13.1 A patient who presents with symptoms suggesting bone cancer or
sarcoma should be referred to a team specialising in the
management of bone cancer and sarcoma, or to a recognised bone
cancer centre, depending on local arrangements.
1.13.2 If a primary healthcare professional has concerns about the
interpretation of a patient’s symptoms and/or signs, a discussion
with the local specialist should be considered.
1.13.3 Patients with increasing, unexplained or persistent bone pain or
tenderness, particularly pain at rest (and especially if not in the
joint), or an unexplained limp should be investigated by the primary
healthcare professional urgently. The nature of the investigations
will vary according to the patient’s age and clinical features.
• In older people metastases, myeloma or lymphoma, as well as
sarcoma, should be considered.
1.13.4 A patient with a suspected spontaneous fracture should be referred
for an immediate X-ray.
1.13.5 If an X-ray indicates that bone cancer is a possibility, an urgent
referral should be made.
1.13.6 If the X-ray is normal but symptoms persist, the patient should be
followed up and/or a repeat X-ray or bone function tests or a referral
The UK guidance on the management of bone sarcomas states the following:
The most common symptom of a primary bone tumour is pain which may be nonmechanical or night pain. The presence of bone pain at night should always be considered to be a “red flag” symptom leading to further investigation. The presence of bone swelling or a soft-tissue mass may occur later. The average duration of symptoms is 3 months although a history of 6 months or longer is not uncommon [13–15]. The presence of pain or a palpable mass arising from any bone should cause concern and lead to further investigation of which a plain X-ray is the first investigation of choice. The presence of any of the following on the X-ray is suggestive, but not diagnostic of a bone tumour and should also lead to further investigation:
- bone destruction,
- new bone formation,
- periosteal swelling,
- soft tissue swelling.
Referral forms for the London Sarcoma Service can be found here.
References UK guidelines for the management of bone sarcomas. NICE referral guidelines for suspected cancer 2005
According to Levesque et al in their excellent book “A clinical guide to Primary Bone Tumours”, the five ways in which patients with musculoskeletal tumours present are:
- A soft tissue mass
- A painless bony mass
- A bone tumour as an incidental finding
- A painful bone lesion
- A pathological fracture
The approach to each clinical scenario has a different emphasis.
Examination of the patient with a musculoskeletal tumour differs a little from a routine examination of a limb. The aim is to look for findings which support your diagnosis.
When examining a mass, you should consider the following:
- depth (superficial to deep fascia or involving/deep to it)
- shape and surfaces
- tethering of overlying skin
- draining lymph nodes
Associated features might include:
- involvement of neurovascular structures
- signs of malignancy elsewhere (eg abdominal masses)
- involvement of an adjacent joint
- other masses elsewhere (eg hereditary multiple exostoses)
Taking a history from a patient with a suspected bone or soft tissue tumour requires a different emphasis from the rest of orthopaedics. Particular features to note include:
- Length of history (is this a preexisting mass that has changed?)
- Rate of growth
- Pain history (site, character, severity, radiation, modifying factors, periodicity – is there mechanical or night pain?)
- Previous surgery at the site of interest (ie is this a local recurrence?)
- History of malignancy (could this be a metastasis?)
- History of a predisposing condition (eg Hereditary Multiple Exostoses)
- Previous radiotherapy
- Family history (think inherited disorders like Li Fraumeni syndrome)
- Symptoms suggestive of malignancy (eg weight loss, lethargy)