Retrospective Cohort Analysis of the Impact of Puberty on Plexiform Neurofibroma Growth in Patients with Neurofibromatosis Type 1 - 08/09/23
Abstract |
Objective |
To assess the hypothesis that plexiform neurofibroma (PN) growth rates increase during puberty.
Study design |
PN growth rates before and during puberty were compared in a retrospective cohort of children with neurofibromatosis type 1 with puberty defined by Tanner staging. Of 33 potentially eligible patients, 25 had adequate quality magnetic resonance imaging for volumetric analysis and were included in ≥1 anchor cohort. Volumetric analysis was performed for all available imaging studies within the 4 years before and after puberty, and before and after 9- and 11-year-old anchor scans. Linear regression was performed to estimate the slope of change (PN growth rate); growth rates were compared with paired t test or Wilcoxon matched-pairs signed rank test.
Results |
There were no significant difference in rates of PN growth in milliliters per month or milliliters per kilogram per month in the prepubertal vs pubertal periods (mean, 1.33 ± 1.67 vs 1.15 ± 1.38 [P = .139] and −0.003 ± 0.015 vs −0.002 ± 0.02 [P = .568]). Percent increases of PN volumes from baseline per month were significantly higher prepubertally (1.8% vs 0.84%; P = .041) and seemed to be related inversely to advancing age.
Conclusions |
Puberty and its associated hormonal changes do not seem to influence PN growth rate. These findings support those previously reported, but from a typical population of children with neurofibromatosis type 1 with puberty confirmed by Tanner staging.
Le texte complet de cet article est disponible en PDF.Keywords : nerve sheath tumor, cancer epidemiology, magnetic resonance imaging
Abbreviations : DNL, MEK, MRI, NCI, NF1, PN
Plan
Presented at the Children's Tumor Foundation Annual Neurofibromatosis Meeting, Philadelphia, PA, June 23, 2022. |
Vol 260
Article 113513- septembre 2023 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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