Every year, eight to ten children are diagnosed with craniopharyngioma. The chance of survival for this rare brain tumor is high. But survival does not always mean cure. In many children, an important hormone center in the brain is damaged by the tumor, with lifelong consequences. That center, the hypothalamus, balances various hormones, regulates satiety, body temperature, day-night rhythms and behavior. Because of the damage, these children's quality of life can decline significantly.
Dr. Hanneke van Santen, pediatric endocrinologist at the Máxima Center and Wilhelmina Children's Hospital (WKZ): ‘The hypothalamus takes care of bodily balance. If this is no longer there, the sense of satiety, for example, becomes unbalanced, so that a child is always hungry. Also, the burning of energy decreases. This causes these children to develop hypothalamic obesity which is measurable through the Body Mass Index, also known as BMI.'
Clear decrease
Together with Dr. Jiska van Schaik, former PhD student in the Tissing group, Van Santen investigated whether centralization of care for children with craniopharyngioma has an effect on BMI. 'We saw a clear difference. One year after treatment, one in three pre-Máxima children had hypothalamic obesity. If we look at the figures after the opening of the Máxima, it is 12%. The difference two years after treatment is even larger: 29% versus 7%.'
The researchers studied data from a total of 87 children. 62 children treated before Máxima opened and 25 children treated at Máxima. They also looked at whether there is a link between the amount of tumor tissue removed during surgery and the risk of hypothalamic obesity. This link, previously thought to exist, was now found to be non-existent. Van Santen: ‘This is probably because we now select the children better for surgery through discussion in a multidisciplinary consultation. Based on this information, the neurosurgeon can determine the most appropriate surgical policy for each child.’
Centralization and collaboration
According to Van Santen, the BMI decrease can be explained by the fact that since centralization, the treating specialists see many more children with this rare condition. 'All healthcare professionals involved now better understand this impactful side effect of the rare craniopharyngioma.'
The children visit several specialists from both the Máxima Center and the WKZ during the consultation hour at the outpatient clinic. For example, they visit the rehabilitation doctor, sleep expert and psychiatrist. Dieticians and physiotherapists are also closely involved. Each child is then discussed by the specialists. 'Thanks to this intensified, multidisciplinary cooperation, we have begun to recognize patterns and have developed so-called interventions with which we can intervene when necessary.' The effect of these interventions is now being investigated further.
In addition, a clinical study is currently taking place. It examines the effectiveness and safety of a drug that stimulates weight loss and suppresses the sense of hunger in children with hypothalamic damage. In follow-up studies, van Santen and her colleagues are also focusing on the other negative consequences of hypothalamic damage: sleep problems, fluctuations in sodium levels and low body temperature.
'With this study, the interventions and follow-up studies, I hope to get another step closer to the mission of the Máxima: to cure every child with cancer with optimal quality of life.'
The results of the study were published in the journal Neuro-oncology: Van Schaik J, Schouten-van Meeteren AYN, Vos-Kerkhof E, Janssens GO, Porro GL, Fiocco M, Bakker B, Tissing WJE, Hoving EW, van Santen HM. Treatment and outcome of the Dutch Childhood Craniopharyngioma Cohort study: First results after centralization of care. Neuro Oncol. 2023 Dec 8;25(12):2250-2261.