CNS & pituitary tumors

Embryonal tumors

Other embryonal tumors

Embryonal tumor with multilayered rosettes



Last author update: 1 November 2016
Last staff update: 22 January 2024 (update in progress)

Copyright: 2003-2024, PathologyOutlines.com, Inc.

PubMed Search: Embryonal tumor with abundant neuropil and true rosettes

Nirupama Singh, M.D., Ph.D.
Page views in 2023: 3,974
Page views in 2024 to date: 690
Cite this page: Singh N. Embryonal tumor with multilayered rosettes. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cnstumorETMR.html. Accessed February 23rd, 2024.
Embryonal tumor with abundant neuropil and true rosettes, C19MC altered
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
  • ICD-10: C71.9 - malignant neoplasm of brain, unspecified

Epidemiology
  • M = F
  • 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 4

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
Ependymoblastoma, C19MC altered
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
    • Embryonal tumor with abundant neuropil and true rosettes
    • Medulloepithelioma
  • References: 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
  • ICD-O: 9478/3 - embryonal tumor with multilayered rosettes C19MC altered

Epidemiology
  • M = F
  • 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
  • Grayish 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
Medulloepithelioma, C19MC not altered
Definition / general
  • CNS embryonal tumor with prominent pseudostratified neuroepithelium that resembles the embryonic neural tube and poorly differentiated neuroepithelial cells
  • Aggressive CNS embryonal tumor with multilayered rosettes (ETMR) and amplicon C19MC altered, C19MC unaltered or 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 coding
  • ICD-10: C71.9 - malignant neoplasm of brain, unspecified

Epidemiology
  • M = F
  • 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 4

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

Positive stains

Negative stains

Molecular / cytogenetics description
Back to top
Image 01 Image 02