Endocrine Abstracts

Childhood-onset craniopharyngiomas are rare intracranial embryonal malformations of the sellar region arising from remnants of Rathke’s pouch that require life-long control and management of the endocrine, ophthalmological and neuropsychological deficits caused by the tumors and their treatment. Craniopharyngiomas show low-grade histological malignancy and frequently affect hypothalamic/pituitary regions and the optic chiasm due to their location. Hypothalamic involvement...

Craniopharyngiomas are partly cystic embryogenic malformations of the sellar and parasellar region. With an overall incidence of 0.5–2.0 new cases/million population per year, ~30–50% of all cases represent childhood craniopharyngioma. Typical manifestations at diagnosis are some combination of headache, visual impairment, polyuria/polydypsia, growth retardation, and significant weight gain. Therapy of choice in patients with favorable tumor localization is complete ...

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: Craniopharyngioma (CP) are the most common sellar tumors in children. Patients often develop excessive weight gain and obesity due to several factors as involvement or damage of the hypothalamus. Previous studies on the weight development in craniopharyngioma patients have shown an increase in weight before and in the first 10 years after diagnosis leading to an impaired quality of life. The long-term weight development in these patients has not been investigated t...

Background: SUB:Pituitary – Clinical (Generously supported by IPSEN)Hypothalamic involvement (HI) resulting in severe obesity is known to have major impact on quality of life in craniopharyngioma (CP) patients. HI is also associated with disturbances of satiety regulation leading to a failure to thrive and weight loss known as diencephalic syndrome (DS). The rate of DS and the outcome of CP patients with DS is unknown.Methods: CP patients h...

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 ...