Prospective Study of Children and Adolescents With Craniopharyngioma

KRANIOPHARYNGEOM 2007 - Multicenter Prospective Study of Children and Adolescents With Craniopharyngioma

German Society for Pediatric Oncology and Hematology GPOH gGmbH, Arbeitsgemeinschaft Pädiatrische Endokrinologie (APE), Deutsche Gesellschaft für Endokrinologie (DGE), Arbeitsgemeinschaft Pädiatrische Radioonkologie (APRO), Deutsche Gesellschafr für Radioonkologie (DEGRO), Deutsche Kinderkrebsstiftung, International Society of Paediatric Oncology

The present investigation is a prospective, multicenter study evaluating craniopharyngioma patients' prognoses following the various currently-practiced therapeutic strategies.Primary goals of the study are to establish quality standards and compare the various therapy strategies with respect to their effectiveness and impact on the quality of life of treated patients. A stratified randomization of two treatment arms will be conducted with respect to timing of postoperative irradiation for the subgroup of patients ≥5 years of age whose tumors are incompletely resected. The researchers will investigate whether an immediate, postoperative irradiation is superior to progression-contingent irradiation based on alterations to quality of life (PEDQOL) from the time randomization is initiated (3rd month post op) to 3 years after randomization. Progression-free survival and overall survival will be examined as closely-related subgoals.Postoperative data will be evaluated via a surveillance study for all complete resection patients as well as for those patients under 5 years of age regardless of their resection grade.

Quality of life, Endocrinology, Oncology, Hypothalamus, Irradiation, Neurosurgery, Pediatric Brain tumors, Randomized trial

Children and adolescents >= 18 years of age new diagnosed with craniopharyngioma >= 5 years of age and with incomplete resected tumor => randomized in two arms: immediate irradiation after surgery

incomplete resection, wait and watch, MRI-controls every 3 months, and irradiation at the time of progression of residual tumor

•Randomized investigation of children (≥ 5 years of age) and adolescent QoL changes (main goal assessment) following incomplete craniopharyngioma resections at primary diagnosis per QoL endpoint scores (PEDQOL "physical functions" domain) measured from 3rd month after surgery for 3 years after randomization; and the progression-free and total survival rates (subgoal assessments) relative to how these rates relate to postoperative irradiation timing (immediate postsurgery irradiation versus wait-and-see approach + progression-contingent irradiation of residual tumor).

Compilation of applied therapy strategies for craniopharyngiomas in children and adolescents using data capture conforming to SIOP brain tumor groups Evaluation of patients' remission status following the various therapy strategies/modalities for craniopharyngiomas using data detection consistent with SIOP brain tumor groups Evaluation of the health status (ophthalmologic, neuropsychologic and endocrine conditions) and health-related QoL of children and adolescents following treatment of craniopharyngiomas using data capture conforming to SIOP brain tumor groups

Inclusion Criteria: Diagnosed with craniopharyngioma for the first time Age at diagnosis 18 years or less of age Agreement from patient's parents or legal guardian as well as the patient Criteria for inclusion in randomization study Histological diagnosis of craniopharyngioma Age at diagnosis 18 years or less of age Age at primary surgery over 5 years of age Incomplete primary resection Reference radiological confirmation of an incomplete resection Agreement from patient's parents or legal guardian as well as the patient Exclusion Criteria: Age at diagnosis over 18 years of age No QoL measurement for randomization (3 months after surgery).

Boekhoff S, Bison B, Eveslage M, Sowithayasakul P, Muller HL. Craniopharyngiomas presenting as incidentalomas: results of KRANIOPHARYNGEOM 2007. Pituitary. 2019 Oct;22(5):532-541. doi: 10.1007/s11102-019-00983-7.

Daubenbuchel AM, Hoffmann A, Eveslage M, Ozyurt J, Lohle K, Reichel J, Thiel CM, Martens H, Geenen V, Muller HL. Oxytocin in survivors of childhood-onset craniopharyngioma. Endocrine. 2016 Nov;54(2):524-531. doi: 10.1007/s12020-016-1084-5. Epub 2016 Sep 1.

Hoffmann A, Warmuth-Metz M, Lohle K, Reichel J, Daubenbuchel AM, Sterkenburg AS, Muller HL. Fusiform dilatation of the internal carotid artery in childhood-onset craniopharyngioma: multicenter study on incidence and long-term outcome. Pituitary. 2016 Aug;19(4):422-8. doi: 10.1007/s11102-016-0722-5.