Puberty and Plexiform Neurofibroma Tumor Growth in Patients with Neurofibromatosis Type I - 20/02/14
, Eva Dombi, MD 2, Ninet Sinaii, PhD, MPH 3, Jessica Sabo, BS 2, Andrea Baldwin, RN 2, Seth M. Steinberg, MD 4, Constantine A. Stratakis, MD, D Sc 1, Brigitte C. Widemann, MD 2Abstract |
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 (cm2/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.
Le texte complet de cet article est disponible en PDF.Keyword : IGF-1, MRI, NF1, PN, PR
Plan
| Supported by the intramural programs of the National Cancer Institute and the Eunice Kennedy Shriver National Institute of Child Health and Human Development. The authors declare no conflicts of interest. |
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| Registered with clinicaltrials.gov: NCT00924196. |
Vol 164 - N° 3
P. 620-624 - mars 2014 Retour au numéro
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