Consequences of Craniopharyngioma Surgery in Children

Context: Childhood craniopharyngioma, a rare embryogenic tumorous malformation of the sellar region, is characterized by survival rates ranging from 91 to 98%. However, quality of survival is frequently impaired due to proximity to optical, pituitary, and hypothalamic structures. Long-term sequelae substantially reduce the quality of life of approximately 50% of long-term survivors, notably extreme obesity owing to hypothalamic involvement and/or surgical-or radiation-induced lesions. Evidence Acquisition and Synthesis: This report reviews the current understanding of diagnostic and treatment options and their consequences on the prognosis and quality of life in patients with childhood craniopharyngioma based on publications from PubMed, Science Citation Index Expanded, EMBASE, and Scopus from the year 1980 onward. Conclusions: Total resection is the treatment of choice in patients with favorable tumor localization, with extreme care taken to preserve hypothalamic-pituitary and optical nerve functions. When tumor localization is unfavorable, i.e. involvement of hypothalamic or optic structures, a limited resection followed by local irradiation is recommended. Optimal timing of recurrence-inhibiting irradiation after incomplete resection is currently under investigation in an international trial. The rarity of the disease, coupled with limited surgical options, dictates that treatment and long-term monitoring of consequences should be confined to experienced multidisciplinary teams. (J Clin Endocrinol Metab 96: 1981-1991, 2011)