Craniopharyngiomas are embryogenic tumorous malformations of the sellar region, presumably derived from Rathke cleft epithelium. With an overall incidence of 0.52 new cases per million population per year, approximately 3050% of all cases represent childhood craniopharyngioma. Typical manifestations at primary diagnosis are headache, visual impairment, polyuria/polydypsia, growth retardation, and weight gain.
Therapy of choice in patients with favorable tumor localization is total resection with the intention to maintain optical nerve and hypothalamic-pituitary functions. In patients with unfavorable tumor localization (hypothalamic tumor involvement), a limited resection followed by local irradiation is recommended.
Survival rates are ranging from 91 to 98%. Recurrences after complete resection and progressions of residual tumor after incomplete resection are anticipated subsequent events after primary surgery. Accordingly, the appropriate time point of irradiation after incomplete resection is currently under investigation in a randomized trial (KRANIOPHARYNGEOM 2007).
Quality of survival is frequently impaired due to the proximity to optical, pituitary and hypothalamic structures. Long-term sequelae substantially reduce the quality of life of ~50% of long-term survivors, notably extreme obesity owing to hypothalamic involvement and/or surgical- or radiation-induced lesions especially of posterior hypothalamic structures.
Initial hypothalamic tumour involvement, especially when both, anterior and posterior areas are involved, has an apriori effect on the clinical course. Therefore, the recommendations are based on recognizing childhood craniopharyngioma as a chronic disease requiring commensurate monitoring and medical resources for treatment and follow-up in order to provide the best lifetime quality of life for the patient.
Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.
Funding: This work was supported, however funding details are unavailable.