Neoplasms in neurofibromatosis 1 are related to gender but not to family history of cancer
Gladstone E. Airewele
Department of Epidemiology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas
Texas Children’s Cancer Center, Baylor College of Medicine, Houston, Texas
Department of Pediatrics, Baylor College of Medicine, Houston, Texas
Search for more papers by this authorAlice J. Sigurdson
Department of Epidemiology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas
Search for more papers by this authorKaren J. Wiley
Department of Epidemiology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas
Search for more papers by this authorBlake E. Frieden
Department of Epidemiology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas
Search for more papers by this authorLauren W. Caldarera
Department of Epidemiology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas
Search for more papers by this authorVincent M. Riccardi
American Medical Consumers, La Crescenta, California
Search for more papers by this authorRichard Alan Lewis
Department of Pediatrics, Baylor College of Medicine, Houston, Texas
Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas
Department of Ophthalmology, Baylor College of Medicine, Houston, Texas
Department of Medicine, Baylor College of Medicine, Houston, Texas
Search for more papers by this authorMurali M. Chintagumpala
Texas Children’s Cancer Center, Baylor College of Medicine, Houston, Texas
Department of Pediatrics, Baylor College of Medicine, Houston, Texas
Search for more papers by this authorJoann L. Ater
Department of Pediatric Hematology and Oncology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas
Search for more papers by this authorSharon E. Plon
Texas Children’s Cancer Center, Baylor College of Medicine, Houston, Texas
Department of Pediatrics, Baylor College of Medicine, Houston, Texas
Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas
Search for more papers by this authorCorresponding Author
Melissa L. Bondy
Department of Epidemiology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas
Department of Epidemiology, Box 189, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030-4095===Search for more papers by this authorGladstone E. Airewele
Department of Epidemiology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas
Texas Children’s Cancer Center, Baylor College of Medicine, Houston, Texas
Department of Pediatrics, Baylor College of Medicine, Houston, Texas
Search for more papers by this authorAlice J. Sigurdson
Department of Epidemiology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas
Search for more papers by this authorKaren J. Wiley
Department of Epidemiology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas
Search for more papers by this authorBlake E. Frieden
Department of Epidemiology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas
Search for more papers by this authorLauren W. Caldarera
Department of Epidemiology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas
Search for more papers by this authorVincent M. Riccardi
American Medical Consumers, La Crescenta, California
Search for more papers by this authorRichard Alan Lewis
Department of Pediatrics, Baylor College of Medicine, Houston, Texas
Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas
Department of Ophthalmology, Baylor College of Medicine, Houston, Texas
Department of Medicine, Baylor College of Medicine, Houston, Texas
Search for more papers by this authorMurali M. Chintagumpala
Texas Children’s Cancer Center, Baylor College of Medicine, Houston, Texas
Department of Pediatrics, Baylor College of Medicine, Houston, Texas
Search for more papers by this authorJoann L. Ater
Department of Pediatric Hematology and Oncology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas
Search for more papers by this authorSharon E. Plon
Texas Children’s Cancer Center, Baylor College of Medicine, Houston, Texas
Department of Pediatrics, Baylor College of Medicine, Houston, Texas
Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas
Search for more papers by this authorCorresponding Author
Melissa L. Bondy
Department of Epidemiology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas
Department of Epidemiology, Box 189, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030-4095===Search for more papers by this authorAbstract
The risk of malignancies among persons with neurofibromatosis 1 (NF1) is higher than in the general population, but the excess risk has not been precisely estimated. The effects of gender and inheritance pattern on cancer risk are unclear. Therefore, we conducted a historical cohort study to determine cancer risk factors by contacting 138 Caucasian NF1 patients originally seen at Baylor College of Medicine (BCM) in Houston between 1978 and 1984. A total of 304 patients of all ethnic groups were evaluated at BCM during this period. We successfully located 173 patients, 138 of who were Caucasian. We computed standardized incidence ratios (SIRs) with the age-, gender-, and time period-specific rates from the Connecticut Tumor Registry for 2,094 person-years of observation (median follow-up = 16 years). Eleven incident tumors were reported. Females were at much higher risk of cancer than males (SIR = 5.6, 95% confidence interval (CI) 2.7–10.3 and SIR = 0.6; 95% CI, 0.0–3.0, respectively). We found no elevated cancer risk in unaffected first-degree relatives, regardless of whether the proband had cancer or not (SIR = 1.1 95% CI, 0.6–1.8 and SIR = 1.0, 95% CI, 0.6–1.5, respectively). Our results suggest that malignancy in the proband is not the result of a modifying gene that has a significant impact on general cancer risk. Genet. Epidemiol. 20:75–86, 2001. © 2001 Wiley-Liss, Inc.
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