Endocrine Abstracts

Background: Craniopharyngiomas (CP) are rare malformational tumors. Clinical presentation and outcome of patients with congenital CP (cCP) are not clear and refer mainly to few case reports in the literature. The aim of this study was to analyze clinical presentation and outcome in patients with cCP.Clinical cases: Seven hundred and nine patients diagnosed with adamantinomatous CP were recruited 1999-2021 in HIT-ENDO, KRANIOPHARYNGEOM 2000 / 2007 / Regis...

Background: Quality of life (QoL) has become a critical component of therapeutic outcomes in the survivors of childhood-onset craniopharyngioma (CP). Visual deficiency adversely affects daily functioning and QoL in childhood CP. This cohort study aimed to report the vision-related QoL in CP patients. Methods: 120 patients with CP were included in this study and prospectively observed. The primary outcome measure was pediatric QoL (PEDQOL score) in patien...

Craniopharyngiomas are rare, embryonic, malformational tumors of the (supra-)sellar region. Due to tumor and/or treatment-related hypothalamic lesions, patients develop morbid obesity. As a major vascular risk factor, obesity leads to reduced 20-year overall survival and a 3- to 19-fold higher cardiovascular mortality after craniopharyngioma compared to the general population. This review studies craniopharyngioma-specific risk factors for vascular damages. Three databases (Pu...

BackgroundData on female fertility, pregnancy, and outcome of offspring after childhood-onset craniopharyngioma (CP) are rare.Study designObservational study on pregnancy rate and outcome of offspring after CP in postpubertal, female patients recruited in KRANIOPHARYNGEOM 2000/2007 since 2000.Results451 CP patients (223 female) have been recruited. 269 CP patients...

Background: Proton beam therapy (PBT) compared to photon-based radiotherapy (XRT) offers the benefit to administer lower radiation doses to critical organs thereby possibly minimizing the risk of sequelae in patients with residual craniopharyngiomas (CP) after hypothalamus-sparing surgery. The validation in large CP patient cohorts is still pending. Methods: Of 290 childhood-onset CP patients included 2007-2019 in the prospective multicenter trial Kranio...

Objective: Hypothalamic syndrome (HS) in childhood is a rare condition. Its epidemiology is not well known because incidence and prevalence are related to very rare underlying diseases. In addition, different criteria for the syndrome are used across studies. Recognizing HS may be difficult, due to its rareness and variety of symptoms. Having diagnostic criteria for signs and symptoms of hypothalamic dysfunction may aid in early recognition and diagnosis, in the reporting and ...

Craniopharyngiomas (CP) are congenital, benign, embryonic malformations which arise from ectoblastic remnants of Rathke’s pouch and are located in the (supra-)sellar region. Many patients show a reduced growth rate and an increased BMI early before the diagnosis of CP. However, it is unknown whether patients with CP present with increased head circumference before diagnosis. A cohort of 90 patients was screened for medical records of their developmental monitoring visits....

Hypothalamic obesity caused by childhood–onset craniopharyngioma results in long–term cardiovascular morbidity. Knowledge about clinical markers and risk factors for cardiovascular morbidity is scarce. A cross–sectional study on transthoracic echocardiographic parameters was performed to determine the associations with clinical and anthropometric parameters in 36 craniopharyngioma patients. BMI correlated with the thickness of interventricular septum in diastole...

BackgroundObesity, cardiovascular disease (CVD), and relapse/progression have impact on prognosis in pediatric brain tumor (BT) patients.MethodsIn a cross-sectional study, we analyzed nuchal skinfold thickness (NST) on MRI follow-up monitoring as a parameter for body composition (BC) and CVD in 177 BT patients (40 WHO grade 1–2 BT; 31 grade 3–4 BT; 106 craniopharyngioma (CP)), and 53 healthy control...

PurposeChildhood-onset craniopharyngiomas (CP) are diagnosed due to clinical symptoms (symCP) or incidentally (incCP). We investigated clinical manifestations and outcome in incCPs and symCPs.MethodsIncCP were discovered in 4 (3m/1f) and symCP in 214 (101m/113f) CP recruited 2007–2014 in KRANIOPHARYNGEOM 2007. Age, sex, height, body mass index (BMI), tumor volume, degree of resection, pre- and postsurgic...