Obesity and metabolic disturbances in adamantinomatous craniopharyngioma patients

An adamantinous craniopharyngioma (CP) is rare benign pituitary tumour often growing invasively and thereby affecting the hypothalamus. The recurrence rate in CP is high and therapy of choice is surgery, followed by cranial radiotherapy (CRT) in about half of the patients. Patients with CP have a 3-19 fold higher cardiovascular mortality in comparison to the general population. The majority of patients have an extended hypopituitarism that needs balanced hormone supplementation. About half of the patient population suffer from hypothalamic damage due to the tumour or operation. These patients suffer from hypothalamic obesity together with increased vascular morbidity and compromised bone health, thirst disorders as well as somnolence and sleep apnea. Cardiovascular risk factors need up front therapy. There is a need for centralising this rare aggressive and difficult tumour to only a few centres and that it will be managed by a multidisciplinary team with experienced neurosurgeons, endocrinologists, neurooncologists, neuroradiologists, and neuroopthamologists for a better prognosis

Mortality and morbidity in adult craniopharyngioma.

A craniopharyngioma (CP) is an embryonic malformation of the sellar and parasellar region. The annual incidence is 0.5-2.0 cases/million/year and approximately 60 % of CP are seen in adulthood. Craniopharyngiomas have the highest mortality of all pituitary tumors. Typical initial manifestations at diagnosis in adults are visual disturbances, hypopituitarism and symptoms of elevated intracranial pressure. The long-term morbidity is substantial with hypopituitarism, increased cardiovascular risk, hypothalamic damage, visual and neurological deficits, reduced bone health, and reduction in quality of life and cognitive function. Therapy of choice is surgery, followed by cranial radiotherapy in about half of the patients. The standardised overall mortality rate varies 2.88-9.28 in cohort studies. Patients with CP have a 3-19 fold higher cardiovascular mortality in comparison to the general population. Women with CP have an even higher risk