Extra-osseous Ewing sarcoma.

Abstract:

BACKGROUND:Clinical data and data on outcome of extra-osseous Ewing tumors are scarce. PROCEDURE:After a search for Ewing tumors in the database of a single institution over a period of 20 years, 16 out of 192 cases were found to have extra-osseous primary tumors. RESULTS:Ages at initial diagnosis ranged from 2.5 to 17 years. Follow-up period ranged from 4 months to 24.8 years (mean 8.4 years). Eleven patients were treated according to protocols for Ewing tumors, while in 4 cases soft tissue protocols were used. In a single patient only surgery was done. Two patients had progressive disease despite chemotherapy; a third patient had only tumor response on the initial 2 chemotherapy courses. All 3 patients with initially metastatic disease died. One patient developed a second malignancy. Overall survival at 5 years was 75%. Event-free survival (EFS) at 5 years was 68%; for nonmetastatic patients 5-year EFS was 83%. CONCLUSION:The authors conclude that nonmetastasized extra-osseous Ewing tumors have a prognosis at least similar to that of osseous Ewing tumors.

journal_name

Pediatr Hematol Oncol

authors

van den Berg H,Heinen RC,van der Pal HJ,Merks JH

doi

10.1080/08880010902855581

subject

Has Abstract

pub_date

2009-06-01 00:00:00

pages

175-85

issue

4

eissn

0888-0018

issn

1521-0669

pii

911096517

journal_volume

26

pub_type

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