High-grade astrocytoma in very young children.

BACKGROUND

High-grade astrocytomas are rare in young children, but have been reported to have a better prognosis than similar tumors in older patients.

PROCEDURE

We retrospectively reviewed the clinical characteristics, survival, and long-term sequelae for patients younger than 3 years old with high-grade astrocytoma, treated at a single institution between 1984 and 2005.

RESULTS

Sixteen patients were included. Histology included anaplastic astrocytoma (n = 9), glioblastoma multiforme (n = 5), and malignant glioma (n = 2). All patients underwent biopsy or resection, followed by chemotherapy. Six patients received scheduled irradiation and six were irradiated at the time of disease progression. Ten patients are alive at a median follow-up of 11.6 years (range, 1.7-21.6 years). 5-year overall survival (OS) was 66.3% (SE 12.2%), and 5-year event-free survival (EFS) was 28.6% (SE 12.1%). Age at diagnosis was a significant predictor of the hazard of death in a Cox model (HR 2.871, 95%CI 1.015-8.123). Gender and histology did not predict OS or EFS. Trends toward improved OS were detected for patients with hemispheric tumors and those undergoing complete resection. All evaluable survivors (n = 9) had some neurocognitive impairment, with estimated overall cognitive ability ranging from significantly delayed to average; all survivors attending school (n = 5) performed below grade level on achievement testing. Seven of nine evaluable survivors had endocrine dysfunction.

CONCLUSIONS

Young children with high-grade astrocytoma have better long-term overall survival than older patients, but recurrence is common, and most children require irradiation. Long-term complications are frequent and often severe.