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Pediatric Gliomatosis Cerebri: A Review of 15 Years
Emilie George1, Allison Settler1, Scott Connors1
1Department of Neurological Surgery, Weill Cornell Medical College, New York, New York, USA.
Insights
Pediatric gliomatosis cerebri, a rare brain tumor, shows survival differences based on age and pathology. Boys and patients aged 10+ with low-grade tumors had longer survival.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Tumor Pathology
Background:
- Gliomatosis cerebri is a rare, aggressive glial tumor with a poor prognosis.
- Distinct differences exist between adult and pediatric gliomatosis cerebri.
- Understanding pediatric gliomatosis cerebri is crucial for improving outcomes.
Abstract:
Gliomatosis cerebri is a rare glial tumor that carries a poor prognosis. Seen in both adults and children, gliomatosis cerebri appears to differ in these populations as with adult versus pediatric glioblastoma. We present 10 children who either presented to the Weill Cornell Medical College or enrolled in the institution's Gliomatosis Cerebri International Registry alongside a cohort of 89 pediatric patients reported in the literature between 2000 and 2014. Age ranged from 4 months to 21 years, with a male to female ratio of 1.71. Median overall survival for patients in the registry cohort was 17 months (n = 10) and for the historic cohort was 13 months (n = 52). Overall survival was analyzed for the combined cohort and was significantly longer when presenting at age ≥ 10 (20 vs 10 months), for boys (18 vs 11 months), and with low-grade pathology (26.5 vs 12 months) but did not vary significantly by treatment approach.
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