hrp0089fc10.4 | Late Breaking | ESPE2018

Hypothalamus Sparing Surgery Improves the Outcome of Patients with Severe Initial Hypothalamic Involvement of Childhood Craniopharyngioma: Results of the Prospective Multinational Trial KRANIOPHARYNGEOM 2007

Bogusz Agnieszka , Boekhoff Svenja , Warmuth-Metz Monika , Calaminus Gabriele , Eveslage Maria , Muller Hermann L

Context: Quality of survival (QoS) is frequently impaired in childhood-onset craniopharyngioma (CP) patients due to sequelae caused by the hypothalamic syndrome. The debate, whether primary hypothalamic involvement (HI) has apriori prognostic impact or surgical hypothalamic lesions (HL) determine outcome, is controversial. Accordingly, we analyzed, whether CP patients at high risk for hypothalamic obesity due to primary HI of anterior and posterior hypothalamic structures bene...

hrp0095p1-535 | Multisystem Endocrine Disorders | ESPE2022

Case Report: HAX1 Mutation/Kostmann Syndrome: A close cooperation between pediatric endocrinologists and hem-oncologists is crucial!

Mayer Isa , Calaminus Gabriele , Schreiner Felix , Nicole Saenger , Dilloo Dagmar , Gohlke Bettina

Purpose: Clinical problems caused by impaired molecular function of inborn errors of immunity (IEI) genes are not limited to the immunological system but can also affect the endocrine system. An autosomal recessive form of severe congenital neutropenia also known as Kostmann syndrome (KS) is caused by mutations in the gene encoding the mitochondrial protein HAX1. Knowing that most endocrine disorders associated with IEI are hypofunction, a close cooperation be...

hrp0082p1-d3-195 | Pituitary | ESPE2014

Childhood Craniopharyngioma: Changes of Treatment Strategies in Multinational Trials During the Last 12 Years

Hoffmann Anika , Warmuth-Metz Monika , Gebhardt Ursel , Pietsch Torsten , Pohl Fabian , Kortmann Rolf-Dieter , Calaminus Gabriele , Muller Hermann L

Background: Despite high survival rates in childhood craniopharyngioma, prognosis is frequently impaired due to sequelae. Radical surgery was the treatment of choice for several decades. However, even at experienced surgical facilities radical surgery can result in hypothalamic disorders such as severe obesity.Objective and hypotheses: We analyzed, whether treatment strategies for childhood craniopharyngioma patients recruited in German studies (KRANIOPH...

hrp0092p1-398 | Pituitary, Neuroendocrinology and Puberty (2) | ESPE2019

Postoperative Quality of Life in Children and Adolescents with Craniopharyngioma – Results of the Prospective Multicenter Trial KRANIOPHARYNGEOM 2007

Eveslage Maria , Calaminus Gabriele , Warmuth-Metz Monika , Kortmann Rolf-Dieter , Pohl Fabian , Timmermann Beate , Schuhmann Martin , Flitsch Jörg , Faldum Andreas , Müller Hermann L.

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 r...