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Genetic Epidemiology of Ewing's Sarcoma

Open (Enrolling)
Ewing Sarcoma, Peripheral Primitive Neuroectodermal Tumor, PNET

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Trial Information

Genetic Epidemiology of Ewing's Sarcoma

PRIMARY OBJECTIVES: I. To determine the association between the length of Ewing sarcoma
breakpoint region 1-Friend leukemia virus integration 1 (EWS-FLI1) fusion protein binding
sites (microsatellites) and risk of Ewing's sarcoma (ES). II. To determine the frequency
and commonality of Caucasian ancestral markers in cases of ES self-identified as
non-Caucasian (African-American, Asian, Hispanic). III. To determine the association
between genomic variants in ES-related genes and hernia development (i.e. the integrin
signaling pathway) and risk of ES. OUTLINE: Genomic DNA is extracted from participants'
saliva samples and analyzed for expression of EWS-FLI1 and other ES-target genes.

Inclusion Criteria:

- The patient is enrolled on ACCRN07 - The patient has a
diagnosis of Ewing Sarcoma (International Classification of Diseases [ICD] code morphology
9260; topography C40.0-C41.9, C76.0-C76.8, C80.9) and is registered with Children's
Oncology Group (COG) by a North American member institution - The patient must be
diagnosed with Ewing sarcoma between December 24, 2007 and December 31, 2015 - The
patient must have at least one biological parent alive and willing to participate - All
questionnaire respondents must understand English or Spanish - Concomitant treatment on
a therapeutic trial is not required

Type of Study:


Study Design:

Time Perspective: Prospective

Outcome Measure:

Main effect of gene polymorphisms

Outcome Description:

Risk ratios (RR) for the main effect of gene polymorphisms will be calculated using log-linear models. RRs and 95% confidence intervals for the gene-environment interaction are calculated by stratifying the likelihood according to case exposure.

Outcome Time Frame:

Up to 5 years

Safety Issue:


Principal Investigator

Joshua Schiffman

Investigator Role:

Principal Investigator

Investigator Affiliation:

Children's Oncology Group


United States: Institutional Review Board

Study ID:




Start Date:

December 2012

Completion Date:

Related Keywords:

  • Ewing Sarcoma
  • Peripheral Primitive Neuroectodermal Tumor
  • PNET
  • Neuroectodermal Tumors
  • Neuroectodermal Tumors, Primitive
  • Sarcoma, Ewing's
  • Neuroectodermal Tumors, Primitive, Peripheral
  • Sarcoma



Children's Oncology GroupArcadia, California  91006-3776