| Title | Extra-osseous Ewing sarcoma. | | Author(s) | van den Berg H, Heinen RC, van der Pal HJ, Merks JH | | Institution | Department of Paediatric Oncology, Emma Children Hospital AMC, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands. h.vandenberg@amc.uva.nl | | Source | Pediatr Hematol Oncol 2009 Jun; 26(4):175-85. | | MeSH | Adolescent
Antineoplastic Agents, Alkylating
Antineoplastic Agents, Phytogenic
Bone Neoplasms
Child
Child, Preschool
Dactinomycin
Female
Follow-Up Studies
Humans
Ifosfamide
Kaplan-Meiers Estimate
Male
Prognosis
Protein Synthesis Inhibitors
Sarcoma, Ewing's
Treatment Outcome
Vincristine
| | 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. | | Language | eng | | Pub Type(s) | Journal Article
| | PubMed ID | 19437320 |
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