Endocrine Abstracts

Craniopharyngiomas are partly cystic embryogenic malformations of the sellar and parasellar region. With an overall incidence of 0.5–2.0 new cases/million population per year, ~30–50% of all cases represent childhood craniopharyngioma. Typical manifestations at diagnosis are some combination of headache, visual impairment, polyuria/polydypsia, growth retardation, and significant weight gain. Therapy of choice in patients with favorable tumor localization is complete resection with specific focus on maintaining functions of the optical nerve and hypothalamic–pituitary axes. In patients whose unfavorable tumor localization makes maintaining hypothalamic functionality surgically challenging, a limited resection followed by local irradiation is recommended. The overall survival rates are high (92%) but occurrences of reduced quality-of-life are also high. Hypothalamic obesity has major negative impact on quality of life during long-term follow-up. Therapeutic options for hypothalamic obesity are very limited. Recurrences after complete resection and progressions of residual tumor after incomplete resection are frequent post-surgical events. Because irradiation is efficient in preventing tumor progression, appropriate timing of post-surgical irradiation is currently under investigation in a randomized multinational trial (KRANIOPHARYNGEOM 2007), which analyzes quality of life as primary endpoint.

Radical surgical strategies especially in patients with hypothalamic involvement are not recommended due to the risk of severe sequelae. In most cases, childhood craniopharyngioma should be recognized as a chronic disease requiring constant monitoring of the clinical and quality of life consequences in order to provide optimal care of surviving patients.