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...

Background: It is well known that both genetic backgroup and lifestyle influence the development of ‘general’ obesity. However, the role of parental body mass index (BMI) on the development of obesity in long-term survivors of childhood-onset craniopharyngioma (CP) is not well understood. This study aimed to analyze the correlation of patients’ BMI at diagnosis, and last visit and parental BMI at CP diagnosis and further explored potential risk factors for obesi...

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....

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.</p...

Background: Adamantinomatous craniopharyngiomas (CP) are rare malformational tumors. The association between age at diagnosis and the outcome, clinical presentation and treatment of pediatric CP patients is not clear. The aim of this cohort study was to determine clinical presentation, outcome and quality of life in CP patients diagnosed at different AaD. Methods: Seven hundred and twenty-two patients diagnosed with CP were recruited 1999-2021 in HIT-End...

Background: Hypothalamic obesity (HO) is defined as abnormal weight gain resulting in severe persistent obesity due to physical, tumor- and/or treatment related damage of the hypothalamus. The HO epidemiology is poorly understood. We developed a database algorithm supporting the standardized identification of tumor/treatment-related HO (TTR-HO) patients.Methods: The algorithm is used to estimate incidence rates of TTR-HO patients in the German healthcare...