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Health condition · Clinically reviewed

Bone and soft tissue cancer, sarcomas need a specialist centre from the very first biopsy.

Rare, diverse and highly treatable when managed at one of the 15 UK supraregional sarcoma centres. From osteosarcoma and Ewing to liposarcoma, leiomyosarcoma, synovial sarcoma and DFSP.

Jump to treatment
A radiographer guides a patient onto the bed of an advanced 3 Tesla MRI scanner in a London imaging suite

Why trust this guide

  • 01

    Sarcoma-specialist voice

    Written to reflect UK practice at the 15 supraregional commissioned sarcoma centres, where all suspected sarcomas should be biopsied and treated.

  • 02

    Sourced from guidance

    Checked against NICE, NHS England Sarcoma Service Specification, ESMO-EURACAN and RCR sarcoma standards.

  • 03

    Current for 2026

    Reflects modern practice on molecular profiling, targeted therapy (nirogacestat, larotrectinib, imatinib for DFSP) and limb-salvage surgery.

Key facts

Sarcoma at a glance.

The essentials, in plain English - the sarcoma families, how the diagnosis is made and where it should be treated.

  • What it is

    A family of rare cancers arising from bone or soft tissue (muscle, fat, vessels, nerves, cartilage). Around 100 histological subtypes, each with its own biology.

  • Bone sarcomas

    Osteosarcoma, Ewing sarcoma, chondrosarcoma, chordoma, giant cell tumour and adamantinoma. Osteosarcoma peaks in adolescence and again after 60.

  • Soft tissue sarcomas

    Liposarcoma, leiomyosarcoma, UPS, synovial sarcoma, MPNST, DFSP, angiosarcoma, rhabdomyosarcoma and others. Adult incidence around 5 per 100,000 per year.

  • Desmoid tumour

    Aggressive fibromatosis is locally aggressive but does not metastasise. See our dedicated guide for CTNNB1-driven disease and nirogacestat.

  • Diagnosis

    Whole-tumour MRI plus core needle biopsy at a specialist sarcoma centre. A poor biopsy compromises limb salvage and must be done by the treating team.

  • Where you are treated

    The UK commissions 15 supraregional sarcoma centres including Royal Orthopaedic Birmingham, Stanmore, Royal Marsden, UCLH, Christie, Newcastle and Oxford.

Why this guide matters

Sarcoma is rare, but the pathway is well-defined.

The three points below shape everything else on this page and are the difference between a limb-sparing cure and a worse outcome.

  • Bone sarcoma families

    Osteosarcoma (adolescents plus Paget-associated older adults), Ewing sarcoma (children and young adults, EWSR1-FLI1), chondrosarcoma (older adults, cartilage), chordoma (skull base and sacrum), giant cell tumour and adamantinoma.

  • Soft tissue sarcoma families

    Liposarcoma, leiomyosarcoma, UPS, synovial sarcoma, MPNST (in NF1), DFSP, angiosarcoma, rhabdomyosarcoma, epithelioid, clear cell and alveolar soft part sarcoma. GIST and desmoid are covered in dedicated guides.

  • The centre is the treatment

    A biopsy performed anywhere other than a supraregional sarcoma centre can seed the tract, contaminate compartments and force amputation. Refer first, biopsy second.

How the diagnosis is made

From first symptom to a specialist plan.

The steps a UK sarcoma centre will normally follow, in order - so you know what to expect and why the sequence matters.

  1. 01

    Recognising

    Recognising the red flags

    Persistent bone pain worse at night, a soft tissue lump larger than 5 cm, deep to fascia, hard or rapidly growing warrants urgent imaging.

  2. 02

    Recognising

    Urgent whole-tumour MRI

    MRI along the full length of the affected bone or compartment, plus chest CT for staging. PET-CT and bone scan are used selectively.

  3. 03

    Recognising

    Referral to a sarcoma centre

    Any suspected primary bone or soft tissue sarcoma should be referred to one of the 15 UK supraregional centres before biopsy.

  4. 04

    Confirming

    Core needle biopsy at the centre

    The biopsy is planned by the treating surgeon so the tract can be excised at definitive surgery. Poor biopsy planning can force amputation.

  5. 05

    Confirming

    Specialist sarcoma pathology

    Immunohistochemistry plus molecular testing for translocations (EWSR1-FLI1 in Ewing, SS18-SSX in synovial, COL1A1-PDGFB in DFSP) and MDM2 amplification in liposarcoma.

  6. 06

    Planning

    Genetics review when relevant

    Li-Fraumeni (TP53), hereditary retinoblastoma (RB1), NF1 (MPNST risk), Werner and Rothmund-Thomson syndromes warrant a clinical genetics referral.

  7. 07

    Planning

    MDT plan and fertility discussion

    Every case is discussed at a specialist sarcoma MDT. Fertility preservation, bone health and psychosocial support are built into the plan.

Typical timeline: urgent MRI within two weeks, biopsy and MDT plan within four to six weeks of specialist referral.

Symptoms

What sarcoma actually feels like.

The signs are subtle and easy to mistake for musculoskeletal injury or a benign lump. The features below are the ones that matter.

  • Persistent deep bone pain

    Pain that wakes you at night, is not linked to activity and persists beyond a few weeks - not typical of a sports injury.

  • Swelling over a bone

    A hard, firm swelling around the knee, shoulder, thigh or pelvis in a young person or older adult with a history of Paget's disease.

  • A soft tissue lump larger than 5 cm

    Any deep, firm, painless or painful lump above the size of a golf ball warrants an urgent ultrasound or MRI - not a wait-and-watch approach.

  • A growing lump

    Any soft tissue mass that is increasing in size, whatever the starting diameter, should be imaged and referred.

  • Pathological fracture

    A fracture through minor trauma, especially at an unusual site, can be the first sign of an underlying bone tumour.

  • Limited joint range

    A stiff, painful shoulder, hip or knee in a young person, without a clear injury, deserves a musculoskeletal opinion.

  • Neurological signs from an MPNST

    In someone with NF1, new pain, rapid growth or neurological deficit in a plexiform neurofibroma raises concern for malignant transformation.

  • Red flag - urgent referral

    Any of the above should trigger a two-week wait sarcoma pathway or direct referral to a supraregional sarcoma centre.

Treatment

How sarcoma is treated in the UK.

Neoadjuvant chemotherapy for bone sarcomas, wide surgical excision with selective radiotherapy for soft tissue, and an expanding toolkit of targeted and immunotherapy options for advanced disease.

  • Sarcoma MDT and specialist centre

    Every suspected sarcoma is managed at one of the 15 UK supraregional centres. The biopsy, surgery and follow-up all sit within that centre.

  • Neoadjuvant chemotherapy

    For osteosarcoma (MAP: methotrexate, doxorubicin, cisplatin) and Ewing sarcoma (VDC/IE), chemotherapy is given before and after surgery.

  • Limb-salvage surgery

    Wide resection with endoprosthetic replacement is standard for most extremity bone sarcomas. Amputation is reserved for tumours where limb salvage is not possible.

  • Wide surgical excision

    For soft tissue sarcomas, wide excision with negative margins is the mainstay. Compartmental surgery is used where anatomy allows.

  • Radiotherapy

    External beam RT, IMRT, brachytherapy and, selectively, proton beam therapy are used pre- or post-operatively to reduce local recurrence.

  • Systemic therapy for advanced disease

    Doxorubicin, ifosfamide, trabectedin, pazopanib (VEGFR TKI), eribulin (liposarcoma) and gemcitabine-docetaxel (leiomyosarcoma) are the mainstays.

  • Targeted therapy

    Imatinib for DFSP, nirogacestat for progressive desmoid, larotrectinib and entrectinib for NTRK-fusion sarcomas, selpercatinib for RET, tazemetostat for INI1-negative epithelioid sarcoma.

  • Surveillance and late-effects care

    Long-term follow-up for 5 to 10 years with imaging, bone health, cardiac review after doxorubicin, fertility support and psychosocial care.

What this guide is based on

The sources behind every claim on this page.

UK and European sarcoma standards, current at the time of last review.

Key references

Guidelines and standards we relied on.

A quiet reminder

This guide is for information, not medical advice.

Your sarcoma team knows your history and imaging in detail and can tell you which parts apply to you. If in doubt, ask to be seen at a specialist centre.

  • NHS England. Sarcoma Service Specification (Adult and Paediatric).

  • NICE. Suspected cancer: recognition and referral (NG12) - bone and soft tissue sarcoma criteria.

  • ESMO-EURACAN-GENTURIS. Soft tissue and visceral sarcomas: clinical practice guidelines.

  • ESMO-EURACAN-GENTURIS-ERN PaedCan. Bone sarcomas: clinical practice guidelines.

  • Sarcoma UK. Patient information and support (sarcoma.org.uk).

  • Bone Cancer Research Trust. Patient resources on primary bone cancer.

Red flags

When a lump or bone pain needs urgent attention.

The features below should trigger urgent imaging and specialist sarcoma referral rather than local excision or watchful waiting.

  • Persistent night bone pain

    Deep bony pain that is not activity-related and disturbs sleep for more than a few weeks needs an X-ray and MRI, especially in adolescents and young adults.

  • Soft tissue lump over 5 cm

    Any deep, growing or hard lump larger than 5 cm should be assumed to be a sarcoma until proven otherwise - urgent MRI and sarcoma referral.

  • Pathological fracture

    A fracture through minor trauma can be the first presentation of osteosarcoma, chondrosarcoma or metastatic disease - imaging before internal fixation.

  • Rapid change in a known lump

    A lipoma or neurofibroma that changes character, becomes painful or grows quickly can herald dedifferentiated liposarcoma or MPNST.

  • NF1 with a changing plexiform

    In neurofibromatosis type 1, new pain, growth or neurological deficit in a plexiform lesion suggests malignant peripheral nerve sheath tumour.

  • Post-radiation angiosarcoma

    A red, bruise-like patch on previously irradiated skin (typically breast) years after treatment can be angiosarcoma - biopsy urgently.

  • Stewart-Treves syndrome

    Long-standing lymphoedema (classically post-mastectomy) developing a purple nodule can indicate lymphangiosarcoma.

  • Do not biopsy locally

    A biopsy planned by a non-sarcoma surgeon can seed the tract, contaminate compartments and force amputation. Refer first, biopsy at the centre.

  • Retroperitoneal mass

    Vague abdominal fullness, back pain or urinary symptoms with a retroperitoneal mass may be liposarcoma or leiomyosarcoma - refer to a specialist retroperitoneal sarcoma centre.

Living with it

A rare cancer, with a well-mapped pathway.

Four things that make the biggest difference from diagnosis through to long-term survivorship - the right centre, the right support, planning for late effects and knowing your surveillance schedule.

A quiet reminder

Where you start matters as much as what you take.

The single biggest determinant of a good sarcoma outcome is being biopsied and treated at a supraregional specialist centre from day one.

  1. 01 Centre

    Be treated at a specialist centre

    Sarcoma outcomes are directly linked to management at a supraregional centre from the point of biopsy onwards. Ask for referral early.

  2. 02 Support

    Use the charities

    Sarcoma UK, the Bone Cancer Research Trust and Christie Sarcoma Support offer helplines, peer support and evidence-based information written for patients.

  3. 03 Late effects

    Plan for the long term

    Fertility preservation, bone health, cardiac follow-up after doxorubicin and second-cancer screening are part of good sarcoma care.

  4. 04 Recurrence

    Know the surveillance schedule

    Follow-up runs for 5 to 10 years with cross-sectional imaging. Isolated lung or soft tissue recurrences can be resected with curative intent in selected cases.

Frequently asked

Everything we get asked about sarcoma.

Quick answers on where to be treated, molecular profiling, surgery and modern targeted options.

  • What is a sarcoma?

    Sarcomas are rare cancers that arise from bone or soft tissue (muscle, fat, cartilage, blood vessels, nerves). They account for around one per cent of adult cancers and are grouped into bone sarcomas (osteosarcoma, Ewing, chondrosarcoma, chordoma) and soft tissue sarcomas (liposarcoma, leiomyosarcoma, UPS, synovial, MPNST, DFSP, angiosarcoma, rhabdomyosarcoma and others).

  • Where should a sarcoma be diagnosed and treated?

    At one of the 15 UK supraregional commissioned sarcoma centres, which include Royal Orthopaedic Birmingham, Royal National Orthopaedic Hospital Stanmore, Royal Marsden, University College Hospital, Christie Manchester, Nottingham, Newcastle, Oxford, Sheffield, Bristol, Leeds, Cambridge, Cardiff, Edinburgh and Glasgow. The biopsy should be planned and performed by the team who will treat the tumour.

  • What are the warning signs of bone or soft tissue cancer?

    Persistent deep bone pain that is worse at night, a bony swelling, a pathological fracture, or a soft tissue lump that is deeper than fascia, larger than 5 cm, hard or growing. Any of these should trigger urgent MRI and referral to a sarcoma centre.

  • What molecular tests are done on a sarcoma?

    Modern sarcoma pathology uses translocations and gene amplifications to confirm the subtype: EWSR1-FLI1 for Ewing sarcoma, SS18-SSX for synovial sarcoma, COL1A1-PDGFB for DFSP, MDM2 amplification for well-differentiated and dedifferentiated liposarcoma, plus NTRK, RET and INI1 testing to identify patients suitable for targeted therapy.

  • What treatments are used?

    Osteosarcoma and Ewing sarcoma need neoadjuvant chemotherapy (MAP or VDC/IE) followed by limb-salvage surgery with endoprosthetic replacement, then adjuvant chemotherapy. Soft tissue sarcomas are treated with wide surgical excision, often with pre- or postoperative radiotherapy. Metastatic disease is managed with doxorubicin, ifosfamide, trabectedin, pazopanib, eribulin, gemcitabine-docetaxel and, in specific subtypes, targeted agents such as imatinib for DFSP, nirogacestat for desmoid, or larotrectinib for NTRK-fusion tumours.

  • What is the difference between a sarcoma and a desmoid tumour?

    Desmoid tumours (aggressive fibromatosis) are locally aggressive but do not metastasise. They are driven by CTNNB1 or APC mutations and are increasingly managed with active surveillance or targeted therapy (nirogacestat) rather than upfront surgery. GISTs are treated as a separate group with imatinib and other tyrosine kinase inhibitors - see our dedicated guides.

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