An infant born with a large subependymal giant cell astrocytoma is reported and 11 cases in the literature are reviewed by researchers at Children’s Hospital, Boston, MA. A 32-year-old woman was referred with an abnormal fetal ultrasound. The ultrasound at 18.7 weeks showed a septal cardiac mass. MRI of the fetal brain at 20.5 weeks gestation showed a mass in the right frontal region at the foramen of Monro. Fetal echocardiogram revealed 2 large intracardiac tumors. Born at 33 weeks gestation, Apgars were 5, 7, and 8, and the infant was intubated because of fetal distress. Skin examination showed 2 hypopigmented macules on the chin. Echocardiogram revealed global biventricular dysfunction, subvalvular aortic stenosis, and multiple large rhabdomyomata. MRI of the brain on day 4 showed multiple cortical tumors and subependymal nodules, and confirmed the right frontal mass diagnosed in utero. Continuous EEG showed electrographic seizures without clinical correlation. Family history for tuberous sclerosis was negative. The patient died at age 12 days. [1]

COMMENT. Subependymal giant cell astrocytoma, typical of tuberous sclerosis, is rare in the neonate.