Background: Craniopharyngioma is an embryonic tumor of low-grade malignancy. Children and adolescents with this diagnosis are analyzed concerning quality of life (QoL) and (progression-free) survival within the project KRANIOPHARYNGEOM 2007.
Methods: The prospective, multi-center project consists of a randomized, unblinded substudy with adaptive design and an observational study. The randomized substudy for incompletely resected patients compares direct postsurgical radiation with radiation at progression. Endpoint is self-assessment of QoL measured by PEDQOL. In explorative analyses, the influence of additional factors was analyzed using linear mixed models.
Results: In the interim analysis of the randomized substudy according to the intention-to-treat approach only marginal differences concerning QoL between the two treatment groups were observed (n=24). The explorative analyses (n=131) show that ant-/ and posterior preoperative hypothalamic involvement and postoperative hypothalamic lesions are associated with decreased QoL. After complete resection, the QoL is lower than with incomplete resection. Radiation, which is often performed due to progression after incomplete resection, is associated with reduced quality of life.
Conclusion: In order to achieve best QoL for children and adolescents with craniopharyngioma, hypothalamus-sparing therapeutic approaches are recommended. Based on the current data, it is not possible to recommend the optimal time for radiotherapy after incomplete resection with regard to QoL.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology