title: Ewing sarcoma-family tumors that arise after treatment of primary childhood cancer.
abstract: BACKGROUND
  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.
authors.affiliation: Department of Hematology-Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105-2794, USA. sheri.spunt@stjude.org
authors.forename: Sheri L
authors.initials: SL
authors.lastname: Spunt
authors.forename: Carlos
authors.initials: C
authors.lastname: Rodriguez-Galindo
authors.forename: Christine E
authors.initials: CE
authors.lastname: Fuller
authors.forename: JoAnn
authors.initials: J
authors.lastname: Harper
authors.forename: Matthew J
authors.initials: MJ
authors.lastname: Krasin
authors.forename: Catherine A
authors.initials: CA
authors.lastname: Billups
authors.forename: Joseph D
authors.initials: JD
authors.lastname: Khoury
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002148:Calmodulin-Binding Proteins / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D009154:Mutation; D016609:Neoplasms, Second Primary / Q000175:diagnosis* / Q000235:genetics* / Q000628:therapy; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins / Q000235:genetics; D012189:Retrospective Studies; D012512:Sarcoma, Ewing / Q000175:diagnosis* / Q000235:genetics* / Q000628:therapy; D014178:Translocation, Genetic; D016896:Treatment Outcome
chemical_list: D002148:Calmodulin-Binding Proteins; C500986:EWSR1 protein, human; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins