Bone sarcomas are rare in childhood, and their presentation can often mimic more benign complaints or chronic musculoskeletal pain. Ewing sarcomas in particular are often diagnosed after a significant delay from the onset of symptoms. At a population level, a long diagnostic delay is not necessarily associated with worse survival , as tumours that display slow growth also tend to be less aggressive. For any specific individual however, a delayed diagnosis can result in a larger tumour that is more difficult to treat. We explore a case of Ewing sarcoma and discuss how the presenting features, approach to imaging and the role of clinician cognitive bias may have led to diagnostic delay.
Ewing sarcoma is treated with chemotherapy and surgery and/or radiotherapy based on the initial site of disease, size of tumour and response to initial treatment. With current UK treatments, overall survival is approximately 70% for localised tumours and up to 20% in those with metastatic disease. Bone sarcomas usually present with deep-seated mechanical bone pain akin to toothache. The pain can be intermittent over the course of days or weeks, but pain occurring at night should be considered a red flag. Swelling may also present. On plain X-ray, bone sarcomas can demonstrate areas of bone destruction, new bone formation, periosteal inflammation and soft tissue swelling, but in some cases the changes are very subtle. Persistent unexplained symptoms require MRI to exclude tumours and detect potential benign causes that are amenable to treatment.
- ewing sarcoma
- chronic musculoskeletal pain
- bone sarcoma
- delayed diagnosis
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Contributors KW and AVR conceived to use this case as a PSICP article. JT wrote the first draft of the article, with specific orthopaedic contributions from AW and RD. AVR and KW have given direction to style and scope. All authors have been involved in refinement of the article and have approved the final draft.
Competing interests None declared.
Provenance and peer review Commissioned; externally peer reviewed.