Surgical management of craniopharyngiomas--experience with a pediatric series

J Pediatr Endocrinol Metab. 2006 Apr:19 Suppl 1:355-66.

Abstract

Because of their peculiar location and the possible related postoperative sequelae, craniopharyngiomas usually present difficult management, especially in children. The authors report on 20 years experience with a pediatric series. Fifty-four consecutive children were operated on. The goal of surgery was total tumor removal. When pre-operative neuroimaging and/ or intra-operative findings suggested more cautious behavior, subtotal/partial excision was performed, followed by radiotherapy only in selected cases. Gross total mass removal was obtained in 78% of patients, subtotal in 17%, and partial in 5%. Recurrence rate was 7% after total removal and 50% after subtotal/ partial excision. Surgical mortality accounted for 3.7% (11% in case of re-operation). After 9 years median follow-up, up to 60% of the surviving patients rely on hormone replacement, 40% present diabetes insipidus, and 18% are obese. All but two patients enjoy a normal social life. Total surgical removal of craniopharyngiomas should be attempted in children whenever possible in order to minimize the risk of recurrence. In case of huge tumors involving the hypothalamus, a more conservative resection should be performed, followed by a 'wait and see' policy or possible radiotherapy.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Craniopharyngioma / mortality
  • Craniopharyngioma / radiotherapy
  • Craniopharyngioma / surgery*
  • Female
  • Humans
  • Infant
  • Magnetic Resonance Imaging
  • Male
  • Neoplasm Recurrence, Local / epidemiology
  • Neurosurgical Procedures*
  • Pituitary Neoplasms / mortality
  • Pituitary Neoplasms / radiotherapy
  • Pituitary Neoplasms / surgery*
  • Radiosurgery
  • Retrospective Studies
  • Survival Analysis
  • Tomography, X-Ray Computed
  • Treatment Outcome