TY - JOUR
T1 - Puberty and plexiform neurofibroma tumor growth in patients with neurofibromatosis type i
AU - Dagalakis, Urania
AU - Lodish, Maya
AU - Dombi, Eva
AU - Sinaii, Ninet
AU - Sabo, Jessica
AU - Baldwin, Andrea
AU - Steinberg, Seth M.
AU - Stratakis, Constantine A.
AU - Widemann, Brigitte C.
N1 - Funding Information:
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.
PY - 2014/3
Y1 - 2014/3
N2 - 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.
AB - 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.
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U2 - 10.1016/j.jpeds.2013.10.081
DO - 10.1016/j.jpeds.2013.10.081
M3 - Article
C2 - 24321536
AN - SCOPUS:84894349570
SN - 0022-3476
VL - 164
SP - 620
EP - 624
JO - Journal of Pediatrics
JF - Journal of Pediatrics
IS - 3
ER -