Ependymoblastoma of the brainstem: MRI findings and differential diagnosis

Pediatr Blood Cancer. 2014 Jun;61(6):1132-4. doi: 10.1002/pbc.24915. Epub 2014 Jan 25.

Abstract

Ependymoblastoma (EBL) is a rare malignant CNS tumor of early childhood, listed as a subgroup of primitive neuroectodermal tumors (PNET) in the 2007 WHO Classification of Tumours of the Central Nervous System. Histologically, EBL can be defined by multilayered, mitotically active "ependymoblastic" rosettes with central lumen as a histological hallmark. The prognosis seems to be far inferior to other embryonal CNS tumors, and known clinical and MRI characteristics of EBL are based on scattered case reports. We present and discuss two uncommon cases of histopathologically confirmed ependymoblastoma that both seem to originate from the brainstem.

Keywords: atypical teratoid/rhabdoid tumor; diffuse intrinsic pontine glioma; embryonal tumor with abundant neuropil and true rosettes; ependymoblastoma; medulloblastoma; primitive neuroectodermal tumor.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Brain Stem Neoplasms / complications
  • Brain Stem Neoplasms / diagnosis
  • Brain Stem Neoplasms / pathology*
  • Brain Stem Neoplasms / surgery
  • Cranial Nerve Diseases / etiology
  • Diagnosis, Differential
  • Diagnostic Errors*
  • Fatal Outcome
  • Female
  • Humans
  • Hydrocephalus / etiology
  • Infant
  • Magnetic Resonance Imaging*
  • Male
  • Medulloblastoma / diagnosis
  • Neoplasm Invasiveness
  • Neuroectodermal Tumors, Primitive / complications
  • Neuroectodermal Tumors, Primitive / diagnosis
  • Neuroectodermal Tumors, Primitive / pathology*
  • Neuroectodermal Tumors, Primitive / surgery
  • Paresis / etiology
  • Pons / pathology*