title: Puberty and plexiform neurofibroma tumor growth in patients with neurofibromatosis type I.
abstract: OBJECTIVE
  To assess the relationship between pubertal progression and change in plexiform neurofibroma (PN) burden over time in pediatric and young adult patients with neurofibromatosis type 1 and PNs.
  
  
  METHODS
  Analyses accounted for sex, age, race, and chemotherapy. Forty-one patients with neurofibromatosis type 1 (15 female and 26 male patients) were studied at the National Institutes of Health. Tanner stage, testosterone, progesterone, estradiol, insulin-like growth factor -1, luteinizing hormone, and follicle-stimulating hormone were assessed. Tumor volume was measured using magnetic resonance imaging and lesion detection software developed locally. Patients were divided into 2 groups based on whether they were actively progressing through puberty (n = 16) or were peripubertal (n = 25) and were followed for an average of 20 months. Tumor growth rates in the puberty and peripubertal group were analyzed for a subset of patients.
  
  
  RESULTS
  There was no statistically significant difference in tumor burden change over time (cm(2)/kg per month) between the pubertal and peripubertal groups (-0.16 ± 0.34 vs 0.03 ± 1.8, P = .31) and in the PN growth rates before and during puberty (P = .90). Change in tumor volume/patient weight/time did not correlate with testosterone change/time in males or estradiol change/time in females.
  
  
  CONCLUSIONS
  These findings support that hormonal changes of puberty do not accelerate PN growth. Additional long-term follow-up of patients is necessary to further characterize the interaction between puberty and tumor growth.
authors: Section on Endocrinology and Genetics, Program on Developmental Endocrinology & Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD.
authors: Urania
authors: U
authors: Dagalakis
authors: Section on Endocrinology and Genetics, Program on Developmental Endocrinology & Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD. Electronic address: lodishma@mail.nih.gov.
authors: Maya
authors: M
authors: Lodish
authors: Pharmacology and Experimental Therapeutics Section, Pediatric Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD.
authors: Eva
authors: E
authors: Dombi
authors: Biostatistics and Clinical Epidemiology Service, National Institutes of Health Clinical Research Center, Bethesda, MD.
authors: Ninet
authors: N
authors: Sinaii
authors: Pharmacology and Experimental Therapeutics Section, Pediatric Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD.
authors: Jessica
authors: J
authors: Sabo
authors: Pharmacology and Experimental Therapeutics Section, Pediatric Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD.
authors: Andrea
authors: A
authors: Baldwin
authors: Biostatistics and Data Management Section, Center for Cancer Research, National Cancer Institute, Bethesda, MD.
authors: Seth M
authors: SM
authors: Steinberg
authors: Section on Endocrinology and Genetics, Program on Developmental Endocrinology & Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD.
authors: Constantine A
authors: CA
authors: Stratakis
authors: Pharmacology and Experimental Therapeutics Section, Pediatric Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD.
authors: Brigitte C
authors: BC
authors: Widemann
mesh_terms: D000293:Adolescent; D002648:Child; D004967:Estrogens / Q000097:blood; D005260:Female; D005640:Follicle Stimulating Hormone / Q000097:blood; D005500:Follow-Up Studies; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000032:analysis; D007986:Luteinizing Hormone / Q000097:blood; D008279:Magnetic Resonance Imaging; D008297:Male; D018318:Neurofibroma, Plexiform / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000473:pathology*; D011374:Progesterone / Q000097:blood; D011446:Prospective Studies; D011627:Puberty; D013739:Testosterone / Q000097:blood
chemical_list: D004967:Estrogens; D013739:Testosterone; D011374:Progesterone; D007334:Insulin-Like Growth Factor I; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone