Childhood Craniopharyngioma – Experiences from the multinational trials HIT-Endo and KRANIOPHARYNGEOM 2000/2007

Craniopharyngiomas are rare embryogenic malformations of the sellar area with low-grade histological malignancy. Despite high survival rates (87 to 95% in recent series), quality of life is frequently impaired in long-term survivors due to sequelae caused by the anatomical proximity of the tumor to the optic nerve, pituitary gland, and hypothalamus. One of the most serious quality of life complications of craniopharyngioma is hypothalamic obesity, a problem that can manifest before and/or after treatment. Novel insights into mechanisms of neuroendocrine satiety regulation and the pathogenic relevance of the autonomous nervous system are expected to facilitate future therapeutic approaches for hypothalamic syndrome. Current treatment options for craniopharyngioma patients suffering from hypothalamic syndrome are limited. A substantial improvement in prognosis of craniopharyngioma will require the development of risk adapted neurosurgical and radiooncological treatment strategies in a multidisciplinary approach. Recent multicenter cooperation in this area (KRANIOPHARYNGEOM 2000/2007) has already led to beneficial results. The consequences of both the surgical treatment and post-surgical management of the disease are as complicated and hypothalamic-intertwined as the tumors themselves. Risk-adapted surgical strategies at initial diagnosis should aim at a maximal degree of resection, respecting the integrity of optical and hypothalamic structures in order to prevent severe sequelae and therein minimize consequences that could negatively exacerbate patient quality of life. Because initial hypothalamic tumor involvement typically has an a priori, life-long effect on the clinical course experienced by the patient, childhood and adult onset craniopharyngioma should be recognized as chronic diseases requiring constant monitoring of the consequences and developing medical resources for treatment in order to provide not only optimal quality of life for patients, but also to garner additional information with the intent of minimizing what at present are severe consequences of both the disease and its treatment.