Childhood craniopharyngioma

Hermann L. Müller

Craniopharyngiomas (CP) are partly cystic embryogenic malformations of the sellar and parasellar region. With an overall incidence of 0.5-2.0 new cases/million population/year, approximately 30-50 % of all cases represent childhood CP. Typical manifestations at diagnosis are headache, visual impairment, polyuria/polydypsia, growth retardation, puberty development disturbances, and significant weight gain. Therapy of choice in children with favorable tumor localization is complete resection with the intention to maintain optic nerve and hypothalamic-pituitary functions. In children with unfavorable tumor localization (hypothalamic involvement), a limited resection followed by local irradiation is recommended. Although overall surgical survival rates are high (92 %), recurrence after complete resection and progression after incomplete resection are typical post-surgical events. Particularly troublesome for the pediatric patient are the disturbances to their pubescent development and overall growth. Accordingly, the appropriate time point of irradiation after incomplete resection is under investigation in a randomized multinational trial (KRANIOPHARYNGEOM 2007). Quality of life is substantially reduced in approximately 50 % of long-term survivors due to sequelae, notably morbid hypothalamic obesity. CP should be recognized as a chronic disease requiring constant monitoring of the early life as well as post-pubescent consequences and appropriate medical resources for treatment in order to provide optimal quality of survival for patients.