Metabolic complications and their mechanisms in patients with craniopharyngioma.

Abstract

After diagnosis of craniopharyngioma, patients frequently develop a rapid weight gain leading to morbid hypothalamic obesity due to disease- and/or treatment-associated hypothalamic lesions. Hypothalamic obesity should be diagnosed and treated in the context of hypothalamic syndrome. Hypothalamic syndrome includes neuroendocrine deficiencies, disruption of circadian rhythm, disturbed hunger-satiety and thirst feelings, temperature dysregulation, and neurocognitive, sleep and psychosocial behavioral problems. Long-term prognosis is frequently impaired by increased risk for metabolic syndrome, cardiovascular problems, severe impairments of health-related quality of life, and premature mortality. Treatment of hypothalamic syndrome is challenging. Recently, an algorithm for personalized, risk-specific treatment of hypothalamic syndrome has been published. Dextro-amphetamines and other central stimulating agents as well as glucagon-like peptide-1 receptor (GLP-1R) agonists may cause weight loss. Bariatric surgery is effective. However, non-reversible procedures are controversial due to ethical and legal considerations in minors. Hypothalamus-sparing treatment strategies and research on novel therapeutic agents for hypothalamic syndrome are warranted.