Primary brain tumors in children under age 3 years

During the period from 1966 to 1996 the authors analyzed the clinicopathological characteristics of 46 cases of histologically verified primary brain tumors with symptomatic onset during the first 3 years of life. The patient group included 27 males and 19 females. There were 14 patients during the first year, 13 during the second year, and 19 during the third year. Supratentorial tumors (60.9%) were more common than infratentorial tumors. Histologically, neuroepithelial tumors predominated. The incidence of ependymal tumors, particularly malignant ones, and of neuronal/mixed neuronal-glial tumors was higher than in previous reports. Congenital brain tumors, those occurring within 2 months after birth, or tumors of dysplastic origin comprised 42.9% of the tumors that developed within 1 year of birth. At the onset, macrocephaly, failure to thrive, and seizures were prominent symptoms or signs in the younger patients. Focal neurological deficits and increased intracranial pressure predominated in the older patients. All but one patient underwent surgical treatment, and 17 patients received adjuvant therapy after surgery. The prognosis was mainly related to the histology of the malignancy. The outcome of medulloblastomas was poor. The quality of life of surviving patients was relatively good, 77.8% having better performance status (PS) than the Eastern Cooperative Oncology Group PS 2.