ESPE Abstracts (2014) 82 P-D-1-3-192

Diencephalic Syndrome in Childhood Craniopharyngioma: Results of German Multicenter Studies on 485 Long-Term Survivors of Childhood Craniopharyngioma

Anika Hoffmanna, Ursel Gebhardta, Anthe Sterkenburga,b, Monika Warmuth-Metzc & Hermann L Müllera


aDepartment of Pediatrics, Klinikum Oldenburg, Oldenburg, Germany; bUMCG University Hospital, Groningen, The Netherlands; cDepartment of Neuroradiology, University Hospital, Würzburg, Germany


Background: Childhood craniopharyngiomas are known to be associated with an increased risk of excessive weight gain and hypothalamic obesity. Atypical clinical manifestations include the development of a diencephalic syndrome (DS) with a failure to thrive or weight loss.

Objective and hypotheses: In a retrospective study we analyzed 21 of 485 childhood craniopharyngioma patients (4.3%) who presented with a low weight (<−2 BMI SDS) at time of diagnosis. 11 of 21 patients were identified with a DS due to proven hypothalamic involvement.

Method: We demonstrate the clinical manifestations of DS and weight development before and after diagnosis in these 11 patients.

Results: First significant differences between patients with low weight at diagnosis and normal weight patients at diagnosis are observed at an age of 5 years. Within the first 2 years after diagnosis, the weight of DS patients and normal weight patients converge to a similar level. Tumor size does not play a role in respect of DS development. Finally, MRI tumor properties of DS patients were compared with MRI scans of obese patients at time of diagnosis. A trend towards a lower rate of infiltrative growth within the hypothalamus might be related to DS patients.

Conclusion: DS is a rare clinical manifestation in childhood craniopharyngioma but should be considered as a differential diagnosis in failure to thrive. DS at the time of diagnosis does not prevent weight gain after diagnosis of a craniopharyngioma with hypothalamic involvement.

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