Extra-osseous Ewing sarcoma

Hendrik van den Berg, Richard C Heinen, Heleen J van der Pal, Johannes H M Merks

Onderzoeksoutput: Bijdrage aan tijdschriftArtikelpeer review

Samenvatting

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.

Originele taal-2Engels
Pagina's (van-tot)175-85
Aantal pagina's11
TijdschriftPediatric hematology and oncology
Volume26
Nummer van het tijdschrift4
DOI's
StatusGepubliceerd - jul. 2009
Extern gepubliceerdJa

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