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Extra-osseous Ewing sarcoma
Pediatric Hematology and Oncology, 05/15/09
van den Berg H et al. - In a trial to assess clinical data and data on outcome of extra-osseous Ewing tumors, it was found that nonmetastasized extra-osseous Ewing tumors have a prognosis at least similar to that of osseous Ewing tumors.
Methods- After a search for Ewing tumors in the database of a single institution over a period of 20 yrs, 16 of 192 cases were found to have extra-osseous primary tumors.
- Ages at initial diagnosis ranged from 2.5 to 17 yrs.
- Follow-up period ranged from 4 mos to 24.8 yrs.
- 11 pts were treated according to protocols for Ewing tumors, while in 4 cases soft tissue protocols were used.
- In a single pt, only surgery was done.
- 2 pts had progressive disease despite chemotherapy; a third patient had only tumor response on the initial 2 chemotherapy courses.
- All 3 pts with initially metastatic disease died.
- 1 pt developed a second malignancy.
- Overall survival at 5 yrs was 75%.
- Event-free survival (EFS) at 5 yrs was 68%; for nonmetastatic pts 5-yr EFS was 83%.
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