CNS tumor
Embryonal tumors
Embryonal tumor with abundant neuropil and true rosettes


Topic Completed: 1 November 2016

Revised: 2 January 2019, last major update November 2016

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PubMed Search: Embryonal tumor with abundant neuropil and true rosettes

Nirupama Singh, M.D., Ph.D.
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Cite this page: Singh N. Embryonal tumor with abundant neuropil and true rosettes. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumorETMR.html. Accessed May 26th, 2020.
Definition / general
  • CNS embryonal tumor with biphasic architecture
  • Aggressive CNS embryonal tumor with multilayered rosettes (ETMR), amplicon C19MC altered and C19MC unaltered or not tested (Acta Neuropathol 2016;131:803)
Essential features
  • Belongs to ETMR, a new subclassification of embryonal tumors
  • Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity
  • Overexpression of LIN28A protein in C19MC altered embryonal tumors
  • Poor prognosis
ICD coding
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
  • Biphasic architecture
  • Dense clusters of small cells with round / polygonal nuclei, scanty cytoplasm and indistinct cell bodies along with large, paucicellular, fibrillary / neuropil like areas along with neoplastic neurocytic and ganglion cells
  • Mitosis and apoptotic bodies in hypercellular area
  • Multilayered rosettes
Positive stains
Negative stains
Molecular / cytogenetics description
  • Amplicon at 19q13.42 is sensitive and specific diagnostic marker for medulloepithelioma with C19MC alteration (Acta Neuropathol 2014;128:279)
  • Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity
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