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Título:
Increased hight and IGF1 serum levels in children with non- neurifibromatosis Type I Gliomas
Autor/es:
CLEMENT FLORENCIA; MARTIN AYELEN; CASTRO SEBASTIÁN; DECH GASTÓN; FERNÁNDEZ MARÍA CELIA; ROPELATO, MARÍA G; BERGADÁ IGNACIO; BALLERINI, MARÍA G; PENNISI PATRICIA
Reunión:
Congreso; 59th Annual Meeting for the European Society for Paediatric Endocrinology; 2021
Resumen:
Introduction: Gliomas are the most common solid tumours during childhood. In children with neurofibromatosis Type 1 (NF1) and optic pathway glioma (OPG), growth hormone excesshas been described. However, this phenomenon has not been reported in children with OPG without NF1. We aimed to describe the growth and IGFs/IGFBP3 levels in a large cohort of paediatricpatients with non-NF1- associated central nervous system (CNS) tumours.Methodology: Study Design: We performed a cross-sectional study in a large cohort of patients younger than 18 years of age with CNS tumours, whose growth was evaluated at the Endocrinology Division of the ?Hospital de Niños Dr. Ricardo Gutiérrez?. Clinical features, biochemical patterns, growth charts and brain imaging were analyzed. The study was performed according to the Declaration of Helsinki and was approved by the Institutional Review Board.Subjects: Inclusion Criteria:We included all patients with CNS tumours studied in our division between 6/2012 and 4/2021, with clinical and biochemical growth evaluation.Exclusion Criteria: Growth hormone deficiency at tumour diagnosis, craniopharyngiomas and other intrasellar tumours, or previously irradiated patients.Methods: anthropometric data, medical and family history were collected from medical records. Height, weight, IGF1, IGFBP3 were expressed as standard deviation scores (SDS) according tolocal references and Tanner status. Delta height to midparental height (ΔHMH: patient´s height - midparental height) was calculated for each patient and expressed as SDS.Results: We studied 92 patients, 49 bearing gliomas and 43 with non gliomas (meduloblastomas, ependymomas, papillomas, germinomas, atypical rabdoid teratoid tumours). We foundthat ΔHMH and IGF1 levels were significantly higher in patients with gliomas vs non gliomas [ΔHMH 0.75 (0.32-1.18) vs -0.13 (-0.48-0.22), p:0.002; SDS IGF1 1.10 (0.77-1.51) vs 0.47 (-0.25-0.88) p:0.02 Mann Whitney test]. There were no differences in IGF1 levels when patients with gliomas were analyzed by tumour localization, malignancy, and hypothalamic injury. Weight andIGFBP3 levels were similar in patients with gliomas compared to those with others tumours. Discussion: Patients with non-NF1 gliomas are taller than expected when compared to those with other CNS tumours, according to midparental height. The higher levels of IGF1 observed in these patients should be highlighted since an important group of gliomas are chronic, non- resectable tumours, with the potential to grow.