title: Intellectual and functional outcome of children 3 years old or younger who have CNS malignancies.
abstract: OBJECTIVE
  To evaluate the impact of tumor location, clinical parameters, and therapy on neurocognitive, neuroendocrine, and functional outcomes in children < or = 3 years old with intracranial CNS malignancies who survived at least 2 years after diagnosis.
  
  
  METHODS
  Records were retrospectively reviewed for 194 children diagnosed from 1985 to 1999 at St Jude Children's Research Hospital (Memphis, TN).
  
  
  RESULTS
  The median age at diagnosis was 1.8 years (range, 0.1 to 3.5 years). Median follow-up was 7.64 years (2.0 to 19.4 years). Tumors were infratentorial (102), diencephalic (53), and hemispheric (39); 47% required ventriculoperitoneal shunts, 36% developed seizure disorders, and 20% developed severe ototoxicity. Therapy included no radiation therapy (RT) in 57 (30%), local RT in 87 (45%), and craniospinal irradiation (CSI) in 49 (25%). Overall survival at 10 years was 78 +/- 4%. In a longitudinal analysis of 126 patients with at least one neurocognitive evaluation (NE), the mean rate of intelligence quotient (IQ) change for patients who received CSI (-1.34 points per year) and local RT (-0.51 points per year) was significantly different from the no RT group (0.91 points per year; P = .005 and P = .036, respectively). Patients with hemispheric tumors had a significantly greater IQ decline (-1.52 points per year) than those with midline tumors (0.59 points per year; P = .038). Among those with NE > or = 5 years after diagnosis, 71.4% of CSI recipients compared with 23% of local RT recipients had IQ less than 70 (P = .021). Patients undergoing CSI were more likely to develop endocrinopathies (P < .0001) and to require special education (P = .0007).
  
  
  CONCLUSIONS
  In young children with CNS tumors, CSI and hemispheric location are associated with significant declines in IQ scores.
authors.affiliation: Department of Hematology-Oncology, St Jude Children's Research Hospital, 332 N Lauderdale, Memphis, TN 38105-2794, USA. maryam.fouladi@stjude.org
authors.forename: Maryam
authors.initials: M
authors.lastname: Fouladi
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Gilger
authors.forename: Mehmet
authors.initials: M
authors.lastname: Kocak
authors.forename: Dana
authors.initials: D
authors.lastname: Wallace
authors.forename: Gray
authors.initials: G
authors.lastname: Buchanan
authors.forename: Cara
authors.initials: C
authors.lastname: Reeves
authors.forename: Nicole
authors.initials: N
authors.lastname: Robbins
authors.forename: Thomas
authors.initials: T
authors.lastname: Merchant
authors.forename: Larry E
authors.initials: LE
authors.lastname: Kun
authors.forename: Raja
authors.initials: R
authors.lastname: Khan
authors.forename: Amar
authors.initials: A
authors.lastname: Gajjar
authors.forename: Raymond
authors.initials: R
authors.lastname: Mulhern
mesh_terms: D017668:Age of Onset; D016543:Central Nervous System Neoplasms / Q000473:pathology* / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000209:etiology*; D004700:Endocrine System Diseases / Q000209:etiology; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007361:Intelligence Tests; D008297:Male; D009483:Neuropsychological Tests; D011379:Prognosis; D012189:Retrospective Studies; D016019:Survival Analysis