Puberty and plexiform neurofibroma tumor growth in patients with neurofibromatosis type i

Urania Dagalakis, Maya Lodish, Eva Dombi, Ninet Sinaii, Jessica Sabo, Andrea Baldwin, Seth M. Steinberg, Constantine A. Stratakis, Brigitte C. Widemann

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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. Study design 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. Conclusion 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.

Original languageEnglish (US)
Pages (from-to)620-624
Number of pages5
JournalJournal of Pediatrics
Volume164
Issue number3
DOIs
StatePublished - Mar 1 2014

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ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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Dagalakis, U., Lodish, M., Dombi, E., Sinaii, N., Sabo, J., Baldwin, A., Steinberg, S. M., Stratakis, C. A., & Widemann, B. C. (2014). Puberty and plexiform neurofibroma tumor growth in patients with neurofibromatosis type i. Journal of Pediatrics, 164(3), 620-624. https://doi.org/10.1016/j.jpeds.2013.10.081