title: Diffuse intrinsic brainstem tumors in neonates. Report of two cases.
abstract: The authors report on 2 newborn infants with the unusual presentation of intrinsic brainstem tumors. Both nondysmorphic, full-term neonates had cranial nerve palsies and hypotonia. Diagnoses of diffuse intrinsic brainstem gliomas were made on the basis of magnetic resonance imaging, which showed large expansive, nonenhancing intrinsic pontine masses. Intrinsic pontine tumors, characteristically seen in school-age children, are most often high-grade gliomas that are almost invariably fatal. However, the microanatomy and natural history of pontine tumors in neonates are unknown. With parental consent, both newborns were treated expectantly with supportive care but died of progressive disease by 2 weeks of age. In one child, postmortem examination revealed a primary brainstem primitive neuroectodermal tumor. The authors conclude that, as in older children, neonatal intrinsic brainstem tumors may be of a highly malignant nature. The rapid tumor progression in both cases indicates that where a diagnostic procedure may pose significant risks, supportive observation can aid in distinguishing malignant from benign tumor growth.
authors.affiliation: Division of Hematology and Oncology, Department of Paediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Niketa C
authors.initials: NC
authors.lastname: Shah
authors.forename: Amit
authors.initials: A
authors.lastname: Ray
authors.forename: Ute
authors.initials: U
authors.lastname: Bartels
authors.forename: James
authors.initials: J
authors.lastname: Rutka
authors.forename: Eric
authors.initials: E
authors.lastname: Bouffet
authors.forename: James
authors.initials: J
authors.lastname: Drake
authors.forename: Cynthia E
authors.initials: CE
authors.lastname: Hawkins
authors.forename: Annie
authors.initials: A
authors.lastname: Huang
mesh_terms: D020295:Brain Stem Neoplasms / Q000209:etiology / Q000473:pathology* / Q000628:therapy*; D017809:Fatal Outcome; D005260:Female; D005910:Glioma / Q000209:etiology / Q000473:pathology* / Q000628:therapy*; D006801:Humans; D007231:Infant, Newborn