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

Research output: Contribution to journalArticle

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 2014
Externally publishedYes

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Plexiform Neurofibroma
Neurofibromatoses
Puberty
Growth
Tumor Burden
Neoplasms
Neurofibromatosis 1
Testosterone
Estradiol
National Institutes of Health (U.S.)
Follicle Stimulating Hormone
Somatomedins
Luteinizing Hormone
Progesterone
Young Adult
Software
Magnetic Resonance Imaging
Pediatrics
Weights and Measures
Drug Therapy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Dagalakis, U., Lodish, M., Dombi, E., Sinaii, N., Sabo, J., Baldwin, 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

Puberty and plexiform neurofibroma tumor growth in patients with neurofibromatosis type i. / Dagalakis, Urania; Lodish, Maya; Dombi, Eva; Sinaii, Ninet; Sabo, Jessica; Baldwin, Andrea; Steinberg, Seth M.; Stratakis, Constantine A.; Widemann, Brigitte C.

In: Journal of Pediatrics, Vol. 164, No. 3, 03.2014, p. 620-624.

Research output: Contribution to journalArticle

Dagalakis, U, Lodish, M, Dombi, E, Sinaii, N, Sabo, J, Baldwin, A, Steinberg, SM, Stratakis, CA & Widemann, BC 2014, 'Puberty and plexiform neurofibroma tumor growth in patients with neurofibromatosis type i', Journal of Pediatrics, vol. 164, no. 3, pp. 620-624. https://doi.org/10.1016/j.jpeds.2013.10.081
Dagalakis, Urania ; Lodish, Maya ; Dombi, Eva ; Sinaii, Ninet ; Sabo, Jessica ; Baldwin, Andrea ; Steinberg, Seth M. ; Stratakis, Constantine A. ; Widemann, Brigitte C. / Puberty and plexiform neurofibroma tumor growth in patients with neurofibromatosis type i. In: Journal of Pediatrics. 2014 ; Vol. 164, No. 3. pp. 620-624.
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