CNS tumor
Embryonal tumors
Embryonal tumor with multilayered rosettes
Embryonal tumor with abundant neuropil and true rosettes, C19MC altered
Author: Nirupama Singh, M.D., Ph.D.
Topic Completed: 1 November 2016
Minor changes: 26 February 2021
Copyright: 2003-2021, PathologyOutlines.com, Inc.
PubMed Search: Embryonal tumor with abundant neuropil and true rosettes
Table of Contents
Definition / general | Essential features | ICD coding | Epidemiology | Sites | Clinical features | Grading | Diagnosis | Radiology description | Case reports | Treatment | Microscopic (histologic) description | Positive stains | Negative stains | Molecular / cytogenetics descriptionCite this page: Singh N. Embryonal tumor with abundant neuropil and true rosettes, C19MC altered. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cnstumorETMR.html. Accessed April 19th, 2021.
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
- Overexpression of LIN28A protein is highly sensitive and specific (Acta Neuropathol 2012;124:875)
Radiology description
- Contrast enhancing large tumor masses on CT and MRI
Case reports
- 33 month old boy with brainstem tumor (J Neurosurg Pediatr 2015;16:291)
Treatment
- Complete surgical removal, radiotherapy and high dose chemotherapy (J Neurooncol 2016;126:99, Childs Nerv Syst 2016;32:299)
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
