Ewing sarcoma is the second most common primary bone cancer in children and adolescents — a small, round, blue-cell tumour driven by the EWS-FLI1 fusion gene. It is aggressive but highly treatable with modern multimodal therapy combining dose-dense chemotherapy and precise local control by surgery and/or radiotherapy.
Ewing sarcoma belongs to the Ewing sarcoma family of tumours and can arise in bone or in soft tissue (extraosseous Ewing's). Microscopically it is composed of small, round, blue cells with a characteristic genetic translocation.
Persistent and often worse at night.
A swelling that grows over weeks rather than months.
Constitutional symptoms that may mimic infection.
Pathological fracture through an unrecognised tumour.
Ewing sarcoma is defined by a recurrent chromosomal translocation. Genetic confirmation is now standard and underpins emerging targeted-therapy trials.
85% of cases have the t(11;22)(q24;q12) chromosomal translocation, creating the EWS-FLI1 fusion gene.
Less commonly involve other ETS-family genes such as ERG.
Tumour cells classically express CD99 and FLI-1.
RT-PCR or FISH for the fusion gene confirms diagnosis and guides research into targeted therapy.
Imaging followed by a planned biopsy at a specialist centre — an improperly placed biopsy can compromise future limb salvage.
Lytic bone destruction with classic 'onion-skin' periosteal reaction.
Defines tumour extent within bone and soft tissue — critical for surgical planning.
Detects pulmonary metastases — the commonest site of spread.
Stages disease and identifies distant bone or marrow metastases.
With CD99/FLI-1 immunohistochemistry and EWS-FLI1 molecular testing.
Elevated LDH often correlates with tumour burden; ALP and ESR may be raised.
Induction chemotherapy, local control by surgery and/or radiotherapy, then consolidation chemotherapy — over 9–12 months in total.
12–14 weeks of dose-dense VDC/IE — vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide & etoposide.
Limb salvage with wide margins in ~95% of cases; amputation reserved for unresectable tumours.
Highly effective local control — used as adjunct or primary therapy where surgery is unsafe.
Continued chemotherapy after local therapy — total treatment duration 9–12 months.
Short and full-length case videos showing how multidisciplinary surgery preserves limbs, joints and function in children with Ewing sarcoma.
A short look at the surgical management and early recovery of a patient with Ewing sarcoma of the pelvis after multidisciplinary care.
Durable disease-free survival and excellent functional outcome years after surgery and chemotherapy for paediatric pelvic Ewing sarcoma.
One of the youngest children in the world to undergo a shoulder-preserving limb salvage procedure for Ewing sarcoma — restoring function while saving the joint.
After wide en bloc resection preserving vital neurovascular structures, the bone defect is reconstructed using techniques tailored to age, site and growth potential. Expendable bones (ribs, scapula, clavicle) often need no reconstruction.
Metallic implant replacing resected bone and adjacent joint.
Donor bone — alone or combined with a prosthesis for durability.
Living bone from the patient's own leg for biological healing.
For growing children — can be lengthened, sometimes non-invasively.
Custom implants, LUMiC or biological options after internal hemipelvectomy.
Ribs, scapula or clavicle may be removed without reconstruction, preserving good function.
Ewing sarcoma is not just a paediatric disease. With expert multidisciplinary care, adults and elderly patients can achieve excellent long-term outcomes.
Wide resection and reconstruction of a bulky humeral Ewing sarcoma in an adult male — preserving the limb despite extensive disease.
One of the oldest patients with Ewing sarcoma to achieve long-term survival after limb-preserving tumor prosthesis reconstruction.
Maya developed persistent shoulder pain at age 16. MRI revealed extraosseous Ewing sarcoma of the proximal humerus, confirmed by EWS-FLI1 fusion testing. She underwent 14 weeks of VDC/IE chemotherapy, wide resection with megaprosthesis reconstruction and postoperative radiotherapy for close margins. After consolidation chemotherapy she regained full shoulder function and remains disease-free at 5 years.
Intensive early surveillance catches recurrences when salvage is most effective.
