CNS tumor
Embryonal tumors
Ependymoblastoma

Author: Nirupama Singh, M.D., Ph.D. (see Authors page)

Revised: 23 November 2016, last major update September 2016

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Ependymoblastoma [title]

Cite this page: Ependymoblastoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumorependymoblastoma.html. Accessed December 7th, 2016.
Definition / General
  • Aggressive CNS embryonal tumor with multilayered rosettes (ETMR) and amplicon C19MC upregulation
  • Rare tumor of children aged < 4 years
  • Other tumors in this group are (a) embryonal tumor with abundant neuropil and true rosettes, (b) medulloepithelioma
  • See: Acta Neuropathol 2016;131:803, Acta Neuropathol 2014;128:305
Essential Features
  • Belongs to ETMR with or without alteration of C19MC, a new subclassification of embryonal tumors
  • Rosettes are frequent and characteristic of embryonal tumors
  • Ependymoblastoma lacks a neuropil-like matrix and ganglion cell elements
  • Overexpression of LIN28A protein in C19MC altered embryonal tumors
  • Poor prognosis
ICD-10 coding
  • 9478 / 3
Epidemiology
  • Equal in males and females
  • Mostly < 2 years of age
Sites
  • Supratentorial in both hemispheres (70%)
  • Infratentorial in cerebellum and brain stem (30%)
Clinical Features
  • Signs and symptoms of increased intracranial pressure (headache, nausea and visual disturbances)
  • Focal neurologic signs (ataxia and weakness)
Diagnosis
Radiology Description
  • Contrast enhancing large tumor masses on CT and MRI
Case Reports
Treatment
Gross Description
  • Well circumscribed
  • Greyish pink with areas of necrosis and hemorrhage
Micro Description
  • Rosettes are frequent and characteristic of ETMR
  • Sheets and clusters of poorly differentiated cells
  • Lacks neuropil-like matrix and ganglion cells
  • Rosettes are intermixed with small to medium sized embryonal cells with high N / C ratio
Micro Images

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Embryonal tumor

Histopathology of ependymoblastoma

Positive Stains
Negative Stains
Molecular / Cytogenetics Description