Sarcoma and bone cancers

The local Sarcoma and Bone Cancer service is provided Regionally by:

Dr A Hennessy
Dr I Phillips

With local support provided by the Cancer & Information Support Service (CISS) ciss@borders.scot.nhs.uk

Sarcomas and primary bone cancers are classed as rare cancers, with bone and connective tissue cancers accounting for less than 1% of all new cancer cases in Scotland across 2021 and 2022.

Connective tissue sarcomas (soft tissue sarcomas):

These can occur at any age, but most often in middle aged and older adults. Around 80% of new cases are diagnosed in people aged 40 years or older.

Bone cancers:

Osteosarcoma is the most common type of primary bone cancer in adolescents but it can occur at any age. They typically present with persistent localised bone pain in the absence of another explanation (e.g. injury, infection or metabolic conditions). They can grow anywhere on the skeleton, although the most common sites are the thigh, upper shin and upper arm bones.

Ewing’s Sarcoma is most common in children and adolescents and has predominant symptoms of persistent pain and swelling of the affected area. Ewing’s Sarcoma can occur in any bone but most commonly starts in the pelvis, ribs, thigh or shoulder bones. It is also possible to occur in the soft tissues of the body.

Chondrosarcoma is found most often in adults aged 30 to 60 years. Clinical presentation is usually a bony mass with pain often as a late feature. Chondrosarcoma is a cancer of cartilage cells within the bone and most commonly occurs at the hips, ribs, upper thigh or shoulder bones.

There is some evidence of inequalities in diagnosis for younger adult patients and those from more deprived backgrounds. Younger et al. (2018) found that young adult sarcoma patients may be more likely to experience delays to diagnosis through misattribution of symptoms and multiple consultations with their GP, whereas elderly patients were more likely to be referred for further investigation. Bacon et al. (2023) noted that young adults, elderly people, and people from more deprived areas were more likely to present via emergency routes.

Who to refer, who not to refer, how to refer

Refer a person with any of the following to the appropriate local secondary care team as a USC:

A soft tissue mass with one or more of the following characteristics:

rapidly increasing in size (over weeks to months)
5cm or more in size (unless long standing – see good practice regarding lipoma)
deep, tethered, fixed or immobile
hard or craggy
fungating
occurring in the site of a previous lump excision or within a previous radiotherapy field

USS suspicious for a soft tissue sarcoma

X-ray suspicious for a primary bone tumour/sarcoma

If the X-ray suggests metastases, myeloma or lymphoma refer as USC to the appropriate local specialty dependent on the most likely primary cancer site. Urgent orthopaedic referral should be considered if there is impending or acute pathological fracture

Primary care management

Connective tissue sarcoma (soft tissue sarcoma):

The most common presenting feature is a lump or mass.

Assess for the following:

Characteristics of the lump – size, change in size (including how quickly), consistency, fixation to other structures, associated skin changes/ulceration and position on the body
Determine if the lump has arisen in the site of a previously excised lump (even if the prior pathology was benign) or area of the body previously exposed to radiotherapy. Both factors increase the risk of the lump being malignant
Any genetic conditions predisposing to sarcoma - see Regional Genetics Centres for advice on such conditions

In some health boards, the next step in investigating a soft tissue lump of concern is a USS – please follow local pathways.

Bone cancer:

Request a USC X-ray of the appropriate body part for any person with unexplained bone pain or tenderness, which is:

Persistent or worsening (over six weeks or more)
Nocturnal or at rest
Interfering with activities of daily living

Blood investigations may be helpful if bone cancer is suspected but should not delay X-ray investigation. Consider the following blood tests: full blood count, renal function, bone profile and myeloma screen. Thyroid, lung, breast, renal and prostate cancers all commonly metastasise to bone and are more common than primary bone cancer, so it may be helpful to consider these in any assessment and investigation of bone pain.

Sarcoma and bone cancers can be missed. It is important to consider further investigation of a person who presents two or more times with a concern about a lump or bone pain with a negative X-ray.

Good Practice:

Clinical features of soft tissue lesions:

The majority of soft tissue lesions referred are benign on ultrasound scan (USS). Masses present for more than 1 year that are asymptomatic and not growing or changing do not require further investigation. Longstanding masses with new growth, pain or other symptoms/changes should be investigated.

Lumps occurring after an episode of definite or recalled trauma are usually self-resolving. Clinical review four to six weeks following the traumatic episode is reasonable, and patients can be discharged with safety netting if the lump is significantly improved or resolved. Persistent or non-resolving masses can be referred for assessment.

Lipoma:

A lipoma is a benign lump, which can affect up to 1% of the population and is most frequent in people aged 40 to 60 years62. It has the following features:

Dome-shaped or egg-shaped lump usually 2-10cm in diameter

May grow slowly over several years

Feels soft and smooth and is easily moved under the skin with the fingers

May have a rubbery or doughy consistency

A lipoma should not be referred based on size alone. A clinical review in four weeks is reasonable for larger lesions. A referral should be made as a USC if there is rapid change in size or development of the other concerning features for a soft tissue lump noted above.

Bone cancers:

Bone cancers of the long bones are usually excluded by normal X-ray, but further investigation may be required for spine, pelvis, ribs or scapula.

Consideration should be given to referral if bone symptoms persist, but the X-ray is normal.

Other considerations:

Treatment for childhood cancer has been shown to increase the risk of subsequent cancers. Specific regimes that increase the risk of sarcoma include those in which there is exposure to alkylating agents (e.g. Cyclophosphamide). Prior treatment should be noted where applicable.

Who to refer, who not to refer, how to refer

Primary care management

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