Childhood Craniopharyngioma

Abstract

Background: Craniopharyngioma are embryogenic malformations of the sellar area. With an overall incidence of 0.5–2 new cases per million population per year, 30–50% of all cases occur in childhood. Overall survival rates are high. However, quality of life (QoL) is substantially reduced in many survivors due to sequelae such as extreme obesity caused by hypothalamic lesions. Methods: Based on retrospective analysis of 306 patients with childhood craniopharyngioma (HIT ENDO), we found that QoL was negatively related to hypothalamic involvement, tumor size and the number of neurosurgical interventions. Results: Irradiation had no significant impact on long-term QoL. The prospective surveillance of 98 patients in KRANIOPHARYNGEOM 2000 revealed frequent and early events in terms of tumor relapse after apparently complete resection (EFS: 0.60 ± 0.09 at 3 years) and tumor progression after incomplete resection (EFS: 0.22 ± 0.09). Conclusion: We conclude that radical surgery in patients with hypothalamic involvement has a major negative impact on long-term QoL. Innovative treatment strategies are warranted to improve QoL in these patients at risk. Accordingly, the appropriate time point of irradiation after incomplete resection will be analyzed in KRANIOPHARYNGEOM 2007.

History before diagnosis in childhood craniopharyngioma: associations with initial presentation and long-term prognosis

“…They show low-grade histological malignancy (WHO 8I) and frequently affect hypothalamic and pituitary regions due to their location. Hypothalamic involvement (HI) of CP, resulting in pathological patterns of eating behavior and obesity, is the most serious risk factor -its aforementioned manifestations impairing prognosis and quality of life in surviving patients (1,3,4,5,6,7,8,9,10,11,12,13,14,15). suspicion of childhood CP in the differential diagnosis process (5).…”

Treatment with Glucagon-Like Peptide-1 Agonist Exendin-4 in a Patient with Hypothalamic Obesity Secondary to Intracranial Tumor

“…Most tumors in this region are craniopharyngiomas [1], although other types of brain tumors, such as the reported case of a hypothalamic germ cell tumor, are observed [2]. Hypothalamic dysfunction is due to tumor infiltration as well as a consequence of surgery or radiotherapy.…”

“…Hypothalamic dysfunction is due to tumor infiltration as well as a consequence of surgery or radiotherapy. Many patients also develop panhypopituitarism and, despite optimal endocrine management, most patients with hypothalamic lesions experience hyperphagia and rapid weight gain resulting in obesity [1,3]. Hypothalamic obesity is common in patients with hypothalamic tumor location, due to damage by the tumor itself and/or its surgical/radiation treatment [1,4,5,6].…”

“…Many patients also develop panhypopituitarism and, despite optimal endocrine management, most patients with hypothalamic lesions experience hyperphagia and rapid weight gain resulting in obesity [1,3]. Hypothalamic obesity is common in patients with hypothalamic tumor location, due to damage by the tumor itself and/or its surgical/radiation treatment [1,4,5,6]. Excessive weight gain generally continues long after treatment is complete [3,7,8,9].…”

Glucagon-Like Peptide-1 Agonist Exendin-4 Leads to Reduction of Weight and Caloric Intake in a Rat Model of Hypothalamic Obesity

“…HO is common, as about 20% of patients with CP are obese at the time of diagnoses, and greater than 50% develop posttreatment obesity [11,34,35,36]. In the current study, we tested Ex4 in our CMHL rat model mimicking metabolic disturbances typically observed in patients with HO.…”