CNS tumor

Embryonal tumors

Embryonal tumor with multilayered rosettes

Ependymoblastoma, C19MC altered

Topic Completed: 1 September 2016

Minor changes: 4 August 2020

Copyright: 2002-2021,, Inc.

PubMed Search: Ependymoblastoma [title]

Nirupama Singh, M.D., Ph.D.
Page views in 2020: 1,341
Page views in 2021 to date: 1,133
Cite this page: Singh N. Ependymoblastoma, C19MC altered. website. Accessed December 6th, 2021.
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 coding
  • 9478 / 3
  • Equal in males and females
  • Mostly < 2 years of age
  • 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)
Radiology description
  • Contrast enhancing large tumor masses on CT and MRI
Case reports
Gross description
  • Well circumscribed
  • Greyish pink with areas of necrosis and hemorrhage
Microscopic (histologic) 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
Positive stains
Negative stains
Molecular / cytogenetics description
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