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

Philip M. S. Simpson,Robin Reid,Daniel Porter

doi:10.1080/00207540500050113

Abstract

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.

Highlights

Ewing’s sarcoma is a rare malignant tumour occurring primarily in bone and not uncommonly in soft tissue
Nineteen cases of upper limb Ewing sarcoma were identified from the database, 14 of whom were male and five of whom were female
One patient lost to follow-up when he returned to the African continent after 6 months disease free six had three-agent chemotherapy

Summary

Introduction

Ewing’s sarcoma is a rare malignant tumour occurring primarily in bone and not uncommonly in soft tissue. Presenting mainly in the second decade of life it has a predilection for the pelvis, the ribs and the diaphyses of long bones. At the time of presentation, approximately 25% of people have radiological metastases. Prior to chemotherapy the relapse rate was approaching 80%, suggesting systemic spread for many people at the time of diagnosis. The treatment of Ewing’s sarcoma currently involves a multidisciplinary approach. The use of four-agent chemotherapy has revolutionised the prognosis in Ewing’s sarcoma,[1,2] but some debate still remains as to whether radiotherapy or surgery for localised lesions is superior in terms of survival [3,4,5,6,7]

Methods

Results

Conclusion