RARE-11. PERINATAL DIFFUSE INFILTRATING BRAINSTEM GLIOMA: CASE REPORT AND REVIEW OF THE LITERATURE

Abstract

Tumors located within the brainstem account for 10-15% of all childhood brain tumors with a mean age of diagnosis between 5-9 years. Neonatal congenital tumors differ from those in older children both in anatomical predilection and histological spectrum. Few perinatal brainstem tumors have been reported in the literature, with the majority being primitive neuroectodermal tumors. We present a review of the literature for all reported cases of perinatal brainstem tumors. We describe the clinical presentation of a male infant born at 38 4/7 weeks by normal spontaneous vaginal delivery to a 32 year-old female. Labor and delivery was induced due to maternal hypertension but was otherwise unremarkable. Apgar scores were 8 and 9 at 1 minute and 5 minutes, respectively. During initial breast feeding in the delivery room the patient began having expiratory grunting, sternal retractions, and desaturations. A MRI obtained on day 4 of life revealed an abnormal mass-like expansion involving the pons and medulla with T2-hyperintensity and no restricted diffusion or contrast enhancement. Family chose to withdraw supportive cares given the grim prognosis and the infant passed. Postmortem exam showed extensive enlargement centered in the pons and rostral medulla with extension into the cerebellar white matter and cerebral peduncles at the mesencephalic level. Histologic sections showed the tumor was consistent with a infiltrating astrocytoma with a significant element of microglia, particularly at the more infiltrative margins. Many of the neonatal brainstem tumors described in the literature are consistent with primitive neuroectodermal tumors, however, our case illustrates that diffuse infiltrating pontine gliomas can also occur in the neonatal period and should be considered on the differential for any brainstem lesion. Evidence suggests that these lesions are different from those occur in older children and may have different genetic aberrations more susceptible to treatment.