title: Family history of cancer and childhood rhabdomyosarcoma: a report from the Children's Oncology Group and the Utah Population Database.
abstract: Relatively little is known about the epidemiology and factors underlying susceptibility to childhood rhabdomyosarcoma (RMS). To better characterize genetic susceptibility to childhood RMS, we evaluated the role of family history of cancer using data from the largest case-control study of RMS and the Utah Population Database (UPDB). RMS cases (n = 322) were obtained from the Children's Oncology Group (COG). Population-based controls (n = 322) were pair-matched to cases on race, sex, and age. Conditional logistic regression was used to evaluate the association between family history of cancer and childhood RMS. The results were validated using the UPDB, from which 130 RMS cases were identified and matched to controls (n = 1300) on sex and year of birth. The results were combined to generate summary odds ratios (OR(s) ) and 95% confidence intervals (CI). Having a first-degree relative with a cancer history was more common in RMS cases than controls (OR(s) = 1.39, 95% CI: 0.97-1.98). Notably, this association was stronger among those with embryonal RMS (OR(s) = 2.44, 95% CI: 1.54-3.86). Moreover, having a first-degree relative who was younger at diagnosis of cancer (<30 years) was associated with a greater risk of RMS (OR(s) = 2.37, 95% CI: 1.34-4.18). In the largest analysis of its kind, we found that most children diagnosed with RMS did not have a family history of cancer. However, our results indicate an increased risk of RMS (particularly embryonal RMS) in children who have a first-degree relative with cancer, and among those whose relatives were diagnosed with cancer at <30 years of age.
authors: Section of Hematology-Oncology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA.
authors: Philip J
authors: PJ
authors: Lupo
authors: Section of Hematology-Oncology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA.
authors: Heather E
authors: HE
authors: Danysh
authors: Section of Hematology-Oncology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA.
authors: Sharon E
authors: SE
authors: Plon
authors: Center for Children's Cancer Research (C3R), University of Utah Health Sciences Center, Salt Lake City, Utah, USA.|Department of Internal Medicine, University of Utah School of Medicine, Salt Lake City, Utah, USA.
authors: Karen
authors: K
authors: Curtin
authors: Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors: David
authors: D
authors: Malkin
authors: Charité, University Hospital Berlin, Germany.
authors: Simone
authors: S
authors: Hettmer
authors: Seattle Children's Hospital, University of Washington, Seattle, Washington, USA.|Fred Hutchinson Cancer Research Center, Seattle, Washington, USA.
authors: Douglas S
authors: DS
authors: Hawkins
authors: Division of Hematology/Oncology, Department of Pediatrics, University of Texas Southwestern Medical Center and Children's Medical Center, Dallas, Texas, USA.
authors: Stephen X
authors: SX
authors: Skapek
authors: Division of Pediatric Epidemiology and Clinical Research, Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota, USA.
authors: Logan G
authors: LG
authors: Spector
authors: Department of Radiation Sciences, Oncology, Umeå University, Umea, Sweden.
authors: Karin
authors: K
authors: Papworth
authors: Department of Radiation Sciences, Oncology, Umeå University, Umea, Sweden.
authors: Beatrice
authors: B
authors: Melin
authors: Department of Mathematics and Statistics, University of New Mexico, Albuquerque, New Mexico, USA.
authors: Erik B
authors: EB
authors: Erhardt
authors: Division of Epidemiology and Biostatistics, Department of Internal Medicine, University of New Mexico, Albuquerque, New Mexico, USA.
authors: Seymour
authors: S
authors: Grufferman
authors: Center for Children's Cancer Research (C3R), University of Utah Health Sciences Center, Salt Lake City, Utah, USA.|Department of Oncological Sciences, Huntsman Cancer Institute, Salt Lake City, Utah, USA.|Department of Pediatrics, University of Utah Health Sciences Center, Salt Lake City, Utah, USA.
authors: Joshua D
authors: JD
authors: Schiffman
mesh_terms: D000293:Adolescent; D000367:Age Factors; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D016208:Databases, Factual; D005190:Family; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D008297:Male; D016017:Odds Ratio; D011159:Population Surveillance; D012208:Rhabdomyosarcoma / Q000453:epidemiology* / Q000209:etiology*; D012307:Risk Factors; D014589:Utah / Q000453:epidemiology
keywords: Childhood cancer; epidemiology; family history; rhabdomyosarcoma; soft tissue sarcoma