Background

Children with suprasellar low grade glioma (LGG) frequently develop problems to maintain their body weight within the normal range, due to hypothalamic dysfunction. Hypothalamic damage may result in the diencephalic syndrome (DS), characterized by underweight or failure to thrive, but also in hypothalamic obesity (HO). Children with suprasellar LGG presenting with DS at young age often develop HO later in life. The underlying pathophysiology for this change in body mass index (BMI) is not understood. Previous hypotheses have focused on the tumor or its treatment as underlying cause. To better understand its etiology, we aimed to relate changes in BMI over time in children with suprasellar LGG presenting with DS to age, tumor progression, treatment, and endocrine function. We hypothesize that the development of HO in children with LGG presenting with DS is related to maturation status of the hypothalamus at time of injury and thus age.

Methods

In this retrospective study, all cases diagnosed in the Netherlands with suprasellar located LGG, currently treated or followed, with a history of DS developing into HO were included.

Results

In total, 13 children were included. Median age at LGG diagnosis was 2.1 years (range 0.4 – 6.0), median BMI SDS was -2.46. The children developed overweight at a median age of 4.6 years (range 2.2 – 9.8). The median total difference in BMI SDS between underweight and obesity was + 6.21 SDS (range 4.2 – 8.7). No association could be found between transition of DS to HO and onset of a pituitary disorder (present in 69.2%, n=9), surgery, chemotherapy, or tumor behavior. Four had developed central precocious puberty (CPP), of whom three while being underweight or normal weight.

Conclusion

The shift from DS to HO in children with hypothalamic LGG seems to be associated with age and not to tumor behaviour, treatment characteristics or pituitary function. Development of CPP in these children was not related to obesity. Our findings indic