Craniopharyngioma - What's next

Abstract

Following the diagnosis of craniopharyngioma, patients frequently experience rapid and excessive weight gain resulting in morbid hypothalamic obesity. This condition is commonly attributed to damage to hypothalamic structures caused either by the tumor itself or its treatment. Hypothalamic obesity should be understood and managed within the broader clinical framework of hypothalamic syndrome, a complex condition characterized by multiple neuroendocrine deficiencies, disruption of circadian rhythms, dysregulation of hunger, satiety and thirst, altered thermoregulation, as well as cognitive, sleep-related, and psychosocial impairments. The long-term outlook for affected individuals is often poor, primarily due to an elevated risk of developing metabolic syndrome, cardiovascular disease, significant reductions in health-related quality of life, and increased risk of early mortality. Management of hypothalamic syndrome remains highly challenging. Recently, a risk-adapted, personalized treatment algorithm has been proposed to guide clinical care. Therapeutic interventions such as dextroamphetamine and other centrally acting stimulants, along with glucagon-like peptide-1 receptor (GLP-1R) agonists, and setmelanotide have shown potential in promoting weight reduction. Bariatric surgery has also demonstrated efficacy; however, the use of irreversible surgical techniques in pediatric populations remains ethically and legally contentious. This report summarizes perspectives of future research and clinical progress in diagnostics, treatment, and follow-up of patients with craniopharyngioma.

Keywords: Hypothalamus; Irradiation; Metabolic syndrome; Neurosurgery; Obesity; Quality of life.