RT Journal Article
SR Electronic
T1 Long-term Outcome of Acral Ewing Sarcoma
JF Anticancer Research
JO Anticancer Res
FD International Institute of Anticancer Research
SP 1377
OP 1380
DO 10.21873/anticanres.15607
VO 42
IS 3
A1 KATHERINE E. MALLETT
A1 MARK J. HEIDENREICH
A1 ZERAHIAH M. JOSEPH
A1 CHLOE COPPER
A1 PETER S. ROSE
A1 STEVEN L. MORAN
A1 MATTHEW T. HOUDEK
YR 2022
UL http://ar.iiarjournals.org/content/42/3/1377.abstract
AB Background/Aim: Ewing sarcoma is a common primary bone tumor, often located in the distal femur or pelvis. Acral Ewing sarcoma of the upper extremity is exceedingly rare. The aim of this study was to review our institution’s experience with the management of rare acral Ewing sarcomas. Patients and Methods: We retrospectively reviewed the records of 10 patients with bony Ewing sarcomas located distal to the elbow joint. The group included 9 male and 1 female patient with a mean age at diagnosis of 20±12 years and a mean follow-up of 19 years. Results: All patients presented with a primary complaint of a painful mass. The most common location was the metacarpal (n=4). Patients were treated with chemotherapy and either surgery (n=7) or definitive radiotherapy (n=3). The mean tumor size and necrosis on the resected specimens were 4±1 cm and 87% (range=30-100%), respectively. There was one case of local progression in a patient treated with definitive radiotherapy, which led to an amputation. Four patients developed metastatic disease, most commonly to the lungs. The 5-year survival free of metastatic disease or death due to disease was 55% and 60%, respectively. Conclusion: Acral Ewing sarcoma is rare. Combined chemotherapy and surgery lead to definitive local control in all patients, with an acceptable functional outcome.