Background: Pituitary function in children with optic chiasm glioma may be impaired.
Objective and hypotheses: We aim to describe the frequency of endocrine abnormalities at diagnosis of the tumor and over the follow-up period in a group of children with chiasmatic glioma and its relation with different variables.
Method: Retrospective study using the records of patients under 14 years old followed for optic chiasm glioma. Sex, age at diagnosis, personal history of type 1 neurofibromatosis (NF), clinical signs and symptoms at presentation, primary treatment of the tumor, endocrine abnormalities at diagnosis and over the follow-up period were recorded. Quantitative variables are expressed as mean±S.D. and compared using Wilcoxon test. Qualitative variables are expressed as proportions and compared using Fisher test.
Results: 14 patients (six women) were included. The range of age at diagnosis was 6 months to 7 years (2.97±2.32 years) and follow-up time was 8.64±3.30 years (range 4.014.0 years). Six patients suffered NF. Reasons of consultation were neuro-ophthalmic signs/symptoms in eight cases and endocrine in three (precocious puberty in all three). Other three were diagnosed subclinically (neuroimaging). Primary treatment for the glioma was needed in eight children, predominating in patients without NF (P=0.02). The proportion of cases with any endocrine disorder increased to 12/14 at the end of the follow-up period: seven children with precocious puberty and five pituitary deficiencies. Hormonal deficiencies were related to history of neuro-ophthalmic signs before age of five (P=0.02) and to primary treatment requirement (P=0.03). On the other hand, precocious puberty was not related to any other variable.
Conclusions: Children with optic chiasm glioma may present endocrine disorders from the time of diagnosis of the tumor and, mainly, along its evolution. Precocious puberty is the most frequent abnormality. Pituitary deficiencies are related to more aggressive tumors (those presenting with neuro-ophthalmic signs before the age of 5 years or requiring primary treatment).
01 - 03 Oct 2015
European Society for Paediatric Endocrinology