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Soft tissue sarcomas make up 88% of sarcoma diagnoses. Each year, there are around 4,500 new cases of soft tissue sarcoma in the UK, which accounts for around 1.2% of all cancer diagnoses. 43% of cases are diagnosed in people over 65.

Presentation: Soft tissue sarcomas usually present as a lump and they can be found anywhere in the body with presenting symptoms relating to where the sarcoma is – for example, a swelling near the lungs may cause a cough or breathlessness.

Types: There are around 70 different types of soft tissue sarcoma and many are very rare. The most common types of soft tissue sarcoma (excluding GIST) are: Leiomyosarcoma (13%), Undifferentiated Sarcoma (13%) and Liposarcoma (11%). The table
compares these different types; although more rare, Rhabdomyosarcoma and Kaposi’s sarcoma have been included as they are more common in different groups of people.

Grade Common demographics Where it develops
Leiomyosarcoma It is more common in adults, particularly over the age of 50, and very rare in people under 20 This starts from the cells in the smooth muscle
Undifferentiated sarcoma It is more common in adults, particularly over the age of 50 This usually forms in the muscles that are attached to bones. They tend to grow quickly and spread to other parts of the body
Liposarcoma It occurs mainly in adults and is very rare in people under the age of 30 This develops from fatty tissue and can grow anywhere in the body, most commonly in the trunk, limbs and in the retroperitoneum
Rhabdomyosarcoma It is more common in children and adolescents, and rare in adults This develops in the skeletal or voluntary muscles of the body
Kaposi’s sarcoma This is caused by the human herpes virus 8 (HHV8).
It is mostly seen in people with an advanced HIV infection. But can also affect other immuno-compromised patients, such as those who have had an organ transplant
This usually affects the skin, lining of the mouth, and organs. It can develop in several parts of the body at the same time

 


Soft Tissue Sarcoma: Anatomical Site

Soft tissue sarcomas can develop anywhere in the body, but there are more common sites based on the type of soft tissue sarcoma.

Anatomical areas of soft tissue percentiles

Source: Cancer Research UK, the world’s leading independent cancer charity dedicated to saving lives through research, influence and information. © Cancer Research UK [2021]. All rights reserved.


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Craig’s story

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Rebecca’s Reflection

Craig’s lump was initially diagnosed and coded as a sebaceous cyst. Each subsequent consulter assumed this diagnosis was correct.

This potentially delayed his diagnosis and highlights the need to be careful about coding and review the diagnosis at each consultation.


Presentation of soft tissue sarcoma

The symptoms of soft tissue sarcoma can vary depending on the size and location of the tumour. Dr Rebecca Leon, GatewayC GP Lead, discussed the typical presentation and the recommended primary care investigations for soft tissue sarcomas with Mr Amit
Kumar, Consultant Orthopaedic and Sarcoma Surgeon.
 

  

Key points for diagnosis:

Presentation Investigation
  • The most common presentation of soft tissue sarcoma is a painful swelling on the trunk or limbs that is not protruding from the skin
  • It is characterised as being larger than 5cm (2 inches) in size, usually painful (but not always), and can increase in size
  • Concerning features of lumps on examination:
    • Semi-solid/hard
    • Protruding
    • Fungating
    • Fixed, or less mobile
  • It is important to take a thorough history of the patient’s symptoms
  • Painless swellings which are increasing in size or have an unusual appearance should always be investigated
  • An ultrasound should be organised in the first instance, with further referral if results are suspicious. However a normal ultrasound does not necessarily exclude sarcoma and patients should be referred on for further assessment
    if their symptoms are persistent or concerning

NB: It is important to be mindful that some patients will experience all, some or none of these symptoms prior to their diagnosis of a soft tissue sarcoma.

Referral

When discussing referrals with patients it is important to ensure they understand why they are being referred. This honesty will help maintain health practitioner-patient trust and potentially minimise the number of DNAs.

All patients with a suspected or confirmed sarcoma should be referred to a sarcoma specialist centre. Data from the National Cancer Registration and Analysis Service has shown that sarcoma survival rates increase when treated at specialist centres. Information
on Sarcoma Specialist Centres in the UK can be found here.


See the table below for NICE NG12 Guidance on how to refer soft tissue sarcomas.

 

Symptoms and specific features Recommendation
Unexplained lump that is increasing in size For adults: Consider an urgent direct access ultrasound scan (to be performed within 2 weeks) [1.11.4]
For children and young people: Consider a very urgent direct access ultrasound (to be performed within 48 hours) [1.11.6]
Ultrasound scan findings that are suggestive of soft tissue sarcoma For adults: Consider a suspected cancer pathway referral (for an appointment within 2 weeks) [1.11.5]
For children and young people: Consider a very urgent referral (for an appointment within 48 hours) [1.11.7]

NB: In practice, younger people (aged 16 to 24) may be referred using either pathway depending on their age and local arrangements.


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Additional resources

Link: Soft tissue sarcoma, Sarcoma UK
Link: Understanding soft tissue sarcoma, Macmillan Cancer Support (2018)
Link: Sarcoma, Manchester Lumps Clinic
Link: Diagnostic Tool – Is it soft tissue sarcoma?, Sarcoma UK (2019)
Link: Sarcoma specialist centres, Sarcoma UK (2021)
Link: Bone and soft tissue sarcoma – Recognition and referral, NICE (2020)
Link: Sarcoma Services (all ages), NHS England (2019)

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