title: Pediatric craniopharyngiomas: classification and treatment according to the degree of hypothalamic involvement.
abstract: OBJECTIVE
  The current treatment of craniopharyngiomas is evolving into one of a multimodal approach in which the aim is disease control and improved preservation of quality of life (QOL). To date, an appropriate classification system with which to individualize treatment is absent. The objectives of this study were to identify preoperative prognostic factors in patients with craniopharyngiomas and to develop a risk-based treatment algorithm.
  
  
  METHODS
  The authors reviewed data obtained in a retrospective cohort of 66 children (mean age 7.4 years, mean follow-up period 7 years) who underwent resection between 1984 and 2001. Postoperative recurrence rates, vision status, and endocrine function were consistent with those reported in the literature. The postoperative morbidity was related to hypothalamic dysfunction. The preoperative magnetic resonance imaging grade, clinically assessed hypothalamic function, and the sugeon's operative experience (p = 0.007, p = 0.047, p = 0.035, respectively) significantly predicted poor outcome. Preoperative hypothalamic grading was used in a prospective cohort of 22 children (mean age 8 years, mean follow-up period 1.2 years) treated between 2002 and 2004 to stratify patients according to whether they underwent gross-total resection (GTR) (20%), complete resection avoiding the hypothalamus (40%), or subtotal resection (STR) (40%). In cases in which residual disease was present, the patient underwent radiotherapy. There have been no new cases of postoperative hyperphagia, morbid obesity, or behavioral dysfunction in this prospective cohort.
  
  
  CONCLUSIONS
  For many children with craniopharyngiomas, the cost of resection is hypothalamic dysfunction and a poor QOL. By using a preoperative classification system to grade hypothalamic involvement and stratify treatment, the authors were able to minimize devastating morbidity. This was achieved by identifying subgroups in which complete resection or STR, performed by an experienced craniopharyngioma surgeon and with postoperative radiotherapy when necessary, yielded better overall results than the traditional GTR.
authors.affiliation: Department of Pediatric Neurosurgery, Hôpital Necker-Enfants Malades, Paris, France.
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Puget
authors.forename: Matthew
authors.initials: M
authors.lastname: Garnett
authors.forename: Alison
authors.initials: A
authors.lastname: Wray
authors.forename: Jacques
authors.initials: J
authors.lastname: Grill
authors.forename: Jean-Louis
authors.initials: JL
authors.lastname: Habrand
authors.forename: Nathalie
authors.initials: N
authors.lastname: Bodaert
authors.forename: Michel
authors.initials: M
authors.lastname: Zerah
authors.forename: Mercia
authors.initials: M
authors.lastname: Bezerra
authors.forename: Dominique
authors.initials: D
authors.lastname: Renier
authors.forename: Alain
authors.initials: A
authors.lastname: Pierre-Kahn
authors.forename: Christian
authors.initials: C
authors.lastname: Sainte-Rose
mesh_terms: D000293:Adolescent; D000465:Algorithms; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003397:Craniopharyngioma / Q000145:classification / Q000175:diagnosis / Q000601:surgery*; D005260:Female; D006801:Humans; D007029:Hypothalamic Neoplasms / Q000145:classification / Q000175:diagnosis / Q000601:surgery*; D007030:Hypothalamo-Hypophyseal System / Q000473:pathology / Q000601:surgery; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000145:classification / Q000175:diagnosis / Q000601:surgery; D009460:Neurologic Examination; D011183:Postoperative Complications / Q000175:diagnosis / Q000209:etiology*; D011379:Prognosis; D011446:Prospective Studies; D011788:Quality of Life; D012189:Retrospective Studies; D012307:Risk Factors