ESPE Abstracts

Although craniopharyngiomas are of low-grade histological dignity and have good prognosis in terms of overall survival, survivors may suffer from devastating consequences caused by hypothalamic damage. Disease and/or treatment-related hypothalamic damage leads to disturbed hunger-satiety and thirst feelings, decreased energy expenditure, behavioral problems, disturbances of circadian rhythm, temperature dysregulation, and pituitary dysfunction. These patients are at great risk for developing hypothalamic obesity and comorbidities leading to premature mortality. Tumor- and/or treatment-associated lesions of specific posterior hypothalamic areas (i.e. area of mammillary bodies and beyond) are risk factors for the development of severe obesity and reduced quality of life during long-term follow-up. A combination of hypothalamus-sparing surgical intervention and protonbeam therapy has been shown to be effective in order to control residual craniopharynioma with less hypothalamic morbidity. Reported pharmacotherapeutic interventions for hypothalamic obesity can be compromised in four categories: stimulants, antidiabetic agents, hypothalamic-pituitary substitution therapy and others, such as oxytocin or octreotide. The reported effects are generally modest and often transient. Currently, there is still no consensus regarding the optimal pharmacotherapy. There are multiple forms of bariatric surgery in hypothalamic obese craniopharyngioma patients reported, such as laparoscopic adjustable gastric banding (LAGB), gastric bypass, Roux-en-Y gastric bypass, sleeve gastrectomy, and biliopancreatic diversion. Of these, Roux-en-Y gastric bypass showed the best results, with in total six studies and fourteen patients included, of whom all showed improvement of their BMI. However, the use of irreversible bariatric procedures in pediatric CP patients is invasive and controversially discussed for medical, ethical and legal reasons.

Conclusions: The late sequelae of CP can be severe, mainly caused by the consequences of hypothalamic damage and may lead to decreased quality of life. Prevention of hypothalamic obesity is most effective, thus hypothalamus-sparing therapeutic approaches should have main focus in these patients.