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EWING SARCOMA OF THE UPPER EXTREMITY – PRESENTING SYMPTOMS, DIAGNOSTIC DELAY AND OUTCOME



Abstract

Aim: The aim of this study was to look at the presenting features, histological grade, size of primary tumour, method of treatment and patient and doctor delays in upper extremity Ewing sarcoma to observe the effects on local recurrence, metastasis and survival.

Methods: 19 patients with upper extremity Ewing sarcoma were identified using the Scottish Bone Tumour Registry which carries clinical, pathological and radiological data on the majority of bone tumours diagnosed in Scotland over the past 50 years.

Results: With increasing tumour Enneking grade at presentation there was a significantly higher mortality (X2=8.0606, p=0.0178). Patients with a higher grade also had an increased trend towards local recurrence (X2=5.1154, p=0.0775). Grade did not seem to influence the occurrence of metastasis. Patients with larger tumours tended to have a higher mortality (50% vs 27% dead at 5 years). All patients presented clinically with pain and all but two complained of some sort of swelling. It was found that there was a trend towards a higher grade in patients presenting with a longer duration of symptoms (X2=4.6269,p=0.0989). No difference in survival was noted between patients undergoing surgery and chemotherapy and patients undergoing radiotherapy and chemotherapy. Disease-free survival was 100% at both 5 and 10 years for Enneking Grade IIA, 56% at 5 and 10 years for Grade IIB and 0% at 5 years for Grade III.

Conclusions: This study re-emphasises the importance of a delay in diagnosis on outcome. Longer symptom duration results in a higher histological grade at presentation. In turn a higher presenting grade is associated with a higher mortality. In agreement with other studies a larger primary tumour correlates with a poorer outcome. Outcomes in terms of survival are comparable for groups treated with adjuvant radiotherapy or surgery.

The abstracts were prepared by Mr Roger Tillman. Correspondence should be addressed to BOOS at the Royal College of Surgeons, 35–43 Lincoln’s Inn Fields, London WC2A 3PN