Ewing's Sarcoma of the Upper Extremity: Presenting Symptoms, Diagnostic Delay and Outcome

PURPOSE To look at the presenting features, Enneking stage, size of primary tumour, method of treatment and patient and doctor delays in upper extremity Ewing's sarcoma to observe the effects on local recurrence, metastasis and survival. PATIENTS AND METHODS Nineteen patients with upper extremity Ewing's sarcoma were identified using the Scottish Bone Tumour Registry over the past 40 years. RESULTS With increasing tumour Enneking stage at presentation there was a significantly higher mortality (P=0.02). Patients with a higher Enneking stage also had an increased trend towards local recurrence (P=0.08). Stage did not 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. There was a trend towards a higher Enneking Stage in patients presenting with a longer duration of symptoms (P=0.1). 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 stage IIA, 56% at 5 and 10 years for stage IIB and 0% at 5 years for stage III. DISCUSSION This study re-emphasises the potential importance of a diagnostic delay on outcome. Longer symptom duration is associated with a higher Enneking stage at presentation. In turn a higher presenting stage results in a higher mortality. Pain and swelling are prominent clinical findings at first presentation in upper extremity Ewing's.