Endocrine Abstracts

Background: Adamantinomatous craniopharyngioma (CP) is a rare embryonal malformation originating from remnants of the craniopharyngeal duct and located in the sellar and/or parasellar area. Overall survival rates are high (92%), but frequently quality of life (QoL) is severely impaired in patients with CP involving hypothalamic structures. Tumor- and/or treatment-related hypothalamic lesions result in disturbances of circadian rhythms including increased daytime sleepiness.

Patients and Methods: After a median follow-up interval of 10 years (range: 1-39 years), 119 patients (63 female / 56 male) with CP, recruited in KRANIOPHARyNGEOM 2000/2007 and KRANIOPHARyNGEOM Registry 2019 between 2000 and 2022, were analysed for daytime sleepiness by Epworth scale (ES) and for QoL by EORTC QLQ-C30 questionnaire.

Results: CP patients (n= 85) with pathologically increased ES score (ES score > 12) had worse QoL (P= 0.003), when compared with CP patients with normal ES score (n= 34). Pathologically increased daytime sleepiness (ES score) was negatively correlated with QoL (r= -0.395; P< 0.001). Surgical hypothalamic lesions (71% of patients) were associated with higher ES scores, wheras such impact could not be observed for presurgical hypothalamic involment of CP (73% of patients).

Conclusion: As increased daytime sleepiness plays an important role for QoL in survivors of CP, treatment of disturbances of circadian rhythms with medication such as central stimulating agents is recommended. For prevention of increased daytime sleepiness, hypothalamus-sparing surgical treatment startegies should be considered as state of the art in patients with CP.