Abstract
Introduction Ewing’s sarcoma family of tumors was first described by James Ewing in 1921. It is a rare tumor and no one center can hope to accrue sufficient numbers over a reasonable period of time to accurately review them.
Objectives A retrospective analysis of all Ewing’s sarcomas of the upper limb from the Scottish Bone Tumor registry (1940–2000), is presented. Attempts have been made to elaborate on prognostic factors, and to document improvements in survival with trends in local therapy over the study period.
Results The registry included 23 patients with Ewing’s sarcoma of the upper limb bones. Age at diagnosis ranged from 7–58 Yrs (mean −21.6 Yrs). 48% of lesions occurred in the second decade and 26 % in the third decade. Male: Female ratio was 2.8: 1. The commonest site involved was the proximal humerus (30%), followed by scapula (21%), radius (17%) and hand bones (13%).Resectable lesions underwent excision and adjuvant radio/chemo therapy. Cumulative 5 year survival for the series was 52%. Forearm and hand lesions which achieved complete excision had best results (75% five year survival). Proximal humeral lesions had 57 % survival. Scapular lesions had the worst prognosis (20% survival). Death was usually due to metastatic disease (diagnosed mean 12mts after initial presentation). Lesions presenting with pathological fractures, or having metastasis at diagnosis had extremely poor survival. There was an increasing use of surgery, and changes to chemotherapy (Euro-Ewing protocol) during the latter part of the study period. There were no toxic deaths reported.
Discussion Ewing’s sarcoma is a rare lesion. Any of the upper limb bones can be involved. Majority of lesions occur within the first 3 decades of life (87%). Survival is highly dependent on initial presentation. The key prognostic factor is the presence of detectable metastasis at diagnosis. Increased use of surgery and newer chemotherapeutic agents have decreased the local relapse rate over the period of study.
Correspondence should be addressed to BOOS at the Royal College of Surgeons, 35 - 43 Lincoln’s Inn Fields, London WC2A 3PN
