CNS tumor
Embryonal tumors
Medulloepithelioma

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

Revised: 1 September 2017, last major update November 2016

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Medulloepithelioma CNS [title]
Cite this page: Singh, N. Medulloepithelioma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumormedulloepithelioma.html. Accessed November 20th, 2017.
Definition / general
  • CNS embryonal tumor with (a) prominent pseudostratified neuroepithelium that resembles the embryonic neural tube and (b) poorly differentiated neuroepithelial cells
  • Aggressive CNS embryonal tumor with multilayered rosettes (ETMR), with (a) amplicon C19MC altered, (b) C19MC unaltered or (c) C19MC not tested
  • Significant proportions of medulloepithelioma have not shown C19MC alterations (Acta Neuropathol 2016;131:803)
Essential features
  • Belongs to ETMR, a new subclassification of embryonal tumors, some of which have C19MC alterations
  • C19MC altered embryonal tumors overexpress LIN28A protein
  • Poor prognosis
ICD-10 coding
  • 88331, 88332
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)
Grading
  • WHO grade IV
Diagnosis
Radiology description
  • Contrast enhancing large tumor masses on CT and MRI
Case reports
Treatment
Microscopic (histologic) description
  • Neoplastic pseudostratified neuroepithelium resembling embryonic neural tube, with papillary, tubular and trabecular arrangements
  • Sheets of poorly differentiated cells with hyperchromatic nuclei and high N/C ratio
  • Multilayered rosettes may be seen
  • Periodic acid-Schiff positive external limiting membrane
  • No cilia or blepharoplasts on luminal surface of tubules
  • Abundant mitotic figures
  • Rarely mesenchymal differentiation and melanin pigment
Microscopic (histologic) images

Images hosted on other servers:
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Neural tube-like strands

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Epithelial structures with brisk mitotic activity

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Highly cellular

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Medulloepithelioma

Positive stains
Negative stains
Molecular / cytogenetics description