Childhood Craniopharyngioma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Background Information About Childhood Craniopharyngioma
Incidence and Presentation
Craniopharyngiomas are relatively rare pediatric tumors, accounting for about 6% of all intracranial tumors in children.[1,2,3] They are believed to be congenital in origin, arising from ectodermal remnants, Rathke cleft, or other embryonal epithelium in the sellar and/or parasellar area. No predisposing factors have been identified.
Quality-of-life issues are important in this group of patients, and are difficult to assess due to various treatment modalities. Whereas intelligence quotient is usually maintained, behavioral issues and memory deficits attributed to the frontal lobe and hypothalamus are common. Other common problems include visual loss, obesity (which can be life threatening), and the almost universal need for life-long endocrine replacement with multiple pituitary hormones.[2,3,4][Level of evidence: 3iiiC] Vasculopathies...
Because craniopharyngiomas occur in the region of the pituitary gland, endocrine function and growth may be affected. Additionally, the close proximity of the tumor to the optic nerves and chiasm may result in vision problems. Some patients present with obstructive hydrocephalus due to tumor obstruction of the third ventricle. Extremely rarely, the tumor may predominate in the posterior fossa with presenting symptoms of headache, diplopia, ataxia, and hearing loss.
Long-term survival for children with craniopharyngioma is generally very good. Regardless of the treatment modality, long-term survival is approximately 85% in children [2,3] with 5- and 10-year overall survival rates greater than 90%.[5,6,7]
Bunin GR, Surawicz TS, Witman PA, et al.: The descriptive epidemiology of craniopharyngioma. J Neurosurg 89 (4): 547-51, 1998.
Karavitaki N, Wass JA: Craniopharyngiomas. Endocrinol Metab Clin North Am 37 (1): 173-93, ix-x, 2008.