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Updated: Aug 15, 2026
Measuring the Functional Abilities of Children Aged 3-6 Years Old with Observational Methods and Computer Tools
Published on: June 20, 2020
Intellectual and functional outcome of children 3 years old or younger who have CNS malignancies
Maryam Fouladi1, Elizabeth Gilger, Mehmet Kocak
1Department of Hematology-Oncology, St Jude Children's Research Hospital, 332 N Lauderdale, Memphis, TN 38105-2794, USA. maryam.fouladi@stjude.org
Insights
Craniospinal irradiation (CSI) and hemispheric tumor location in young children with central nervous system (CNS) malignancies are linked to significant neurocognitive declines, impacting intelligence quotient (IQ) scores over time.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Child Neurology
Background:
- Intracranial central nervous system (CNS) malignancies in children under 3 years old present unique challenges.
- Long-term outcomes including neurocognitive, neuroendocrine, and functional status are critical for this population.
- Understanding the impact of tumor location and therapy is essential for improving survival and quality of life.
Purpose of the Study:
- To assess the long-term effects of tumor location, clinical factors, and treatment modalities on neurocognitive and functional outcomes.
- To evaluate neuroendocrine sequelae in young survivors of intracranial CNS tumors.
- To identify risk factors associated with adverse outcomes in pediatric CNS malignancy survivors.
Main Methods:
- Retrospective review of 194 children diagnosed with intracranial CNS malignancies between 1985 and 1999.
- Analysis of neurocognitive evaluations, including intelligence quotient (IQ) changes over time.
- Evaluation of treatment exposures, including radiation therapy (RT) and craniospinal irradiation (CSI), and tumor location (infratentorial, diencephalic, hemispheric).
Main Results:
- Craniospinal irradiation (CSI) and local radiation therapy (RT) were associated with significant declines in IQ scores compared to no RT.
- Hemispheric tumors showed a greater decline in IQ compared to midline tumors.
- CSI recipients were more likely to develop endocrinopathies and require special education.
Conclusions:
- Craniospinal irradiation (CSI) and hemispheric tumor location are significant predictors of neurocognitive decline in young children with CNS tumors.
- Early-life CNS malignancies and their treatments can lead to lasting neurodevelopmental deficits.
- Further research is needed to optimize treatment strategies and mitigate long-term sequelae.
Purpose:
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.
Patients And 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).
Conclusion:
In young children with CNS tumors, CSI and hemispheric location are associated with significant declines in IQ scores.
