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Ewing sarcoma-family tumors that arise after treatment of primary childhood cancer Spunt SL; Rodriguez-Galindo C; Fuller CE; Harper J; Krasin MJ; Billups CA; Khoury JDCancer 2006[Jul]; 107 (1): 201-6BACKGROUND: Unlike osteosarcoma, the Ewing sarcoma family of tumors (ESFT) has rarely been reported as secondary malignant neoplasms after treatment of childhood cancer. ESFT arising as a second cancer was reviewed and characterized at our childhood cancer center. METHODS: A retrospective review was undertaken of 11,183 patients age <21 years who were treated for a primary cancer between March 1962 and December 2003 at St. Jude Children's Research Hospital. All cases of ESFT were confirmed to have a rearranged EWS gene. RESULTS: Six cases of ESFT (1.3% of 479 second cancers) were identified in patients previously treated for lymphoma (n = 3), leukemia (n = 1), retinoblastoma (n = 1), or Wilms tumor (n = 1). None of these patients had a family history suggestive of a familial cancer syndrome. The median time between diagnosis of primary cancer and diagnosis of ESFT was 5.9 years (range, 3.1-18.3 years). ESFT occurred in typical anatomic locations: rib (n = 2), chest wall soft tissues (n = 2), pelvis (n = 1), and extremity (n = 1). One tumor arose at the margin of a previous radiotherapy field and 1 arose distant from previous radiotherapy fields; all other patients had not received radiotherapy. Three patients are alive at the time of this report, including 2 whose ESFT was diagnosed more than 8 years ago. CONCLUSIONS: ESFT occurs rarely after treatment of a primary cancer during childhood, and most cases do not appear to be related to radiation therapy. Long-term survival can be achieved in some patients, and therefore secondary ESFT should be treated with curative intent.|Adolescent[MESH]|Adult[MESH]|Age of Onset[MESH]|Calmodulin-Binding Proteins/genetics[MESH]|Child[MESH]|Child, Preschool[MESH]|Humans[MESH]|Infant[MESH]|Mutation[MESH]|Neoplasms, Second Primary/*diagnosis/*genetics/therapy[MESH]|RNA-Binding Protein EWS[MESH]|RNA-Binding Proteins/genetics[MESH]|Retrospective Studies[MESH]|Sarcoma, Ewing/*diagnosis/*genetics/therapy[MESH]|Translocation, Genetic[MESH]|Treatment Outcome[MESH] |
