Ichelle van Roessel1, Sanne Hulsmann2, Netteke Schouten-van Meeteren3, Eelco Hoving3, Geert Janssens3, Martine Raphael4, Evelien de Vos-Kerkhof4, Boudewijn Bakker5, Wim Tissing5, Hanneke van Santen6
(1) Princess Máxima Center / Wilhelmina Children\’s Hospital, Utrecht, The Netherlands. (2) Princess Máxima Center / Wilhelmina Children\’s Hospital, Utrecht, The Netherlands. (3) Princess Máxima Center, Utrecht, The Netherlands. (4) Emma Children’s Hospital, Academic Medical Center, Amsterdam, The Netherlands. (5) Princess Máxima Center / Wilhelmina Children\’s Hospital, Utrecht, The Netherlands. (6) Princess Máxima Center / Wilhelmina Children\’s Hospital, Utrecht, The Netherlands.
Background:
Hypothalamic dysfunction (HD) following treatment for a suprasellar brain tumor in childhood may have devastating consequences, with hypothalamic syndrome (HS) as the most severe presentation of HD. Morbid obesity, and pituitary dysfunction are readily recognized as consequences of HD, however HD may also lead to overweight, hypothermia, sleep- and/or behavioral problems. In our experience, symptoms of HD differ and HD as an underlying cause for weight gain or fatigue may be overlooked. Here, we aimed to systematically describe the multifaceted character of acquired HD.
Materials / methods:
Retrospective assessment of children diagnosed with a suprasellar low-grade glioma (LGG), craniopharyngioma (cCP), or suprasellar intracranial germ cell tumor (CNS GCT) in the Netherlands between 2003-2023.
Results:
At diagnosis, overweight or obesity was present in 63 of all patients (18·8%), of whom 45 (71·4%) had an adequate pituitary function.
Signs of hypothalamic dysfunction were found in 273 of 336 children (81·3%) with a suprasellar brain tumor. At follow-up, HS was present in 38·1% of patients. In patients with HS, 42 different faces of hypothalamic dysfunction were observed, ranging from morbid obesity with or without pituitary dysfunction to overweight with sleep disorder, behavioral problems and/or temperature dysregulation.
Conclusion:
Hypothalamic dysfunction does not have a uniform presentation, but has many different clinical faces and concerns a far broader perspective than hypothalamic obesity. Obesity can be caused by a suprasellar tumor, even if the pituitary function is still intact. Recognition of the different faces of hypothalamic dysfunction is important to reduce delay in diagnosis of a suprasellar tumor as well as for more personalized management during follow-up.