Rare and aggressive: A case of embryonal tumor with multilayered rosettes (ETMR) in a young child

Embryonal tumors with multilayered rosettes (ETMR) are rare, highly aggressive brain neoplasms predominantly affecting children under 3 years of age. In the 2021 WHO Classification of Tumors of the Central Nervous System, ETMRs are classified as grade IV tumors, previously considered separate entiti...

Full description

Saved in:
Bibliographic Details
Main Authors: Oukassem siham, Abourak Chaimae, Khatibi Wafa, Belkouchi Lina, El Haddad Siham, Allali Nazik, Chat Latifa
Format: Article
Language:English
Published: Elsevier 2025-10-01
Series:Radiology Case Reports
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S1930043325005874
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1849721137910513664
author Oukassem siham
Abourak Chaimae
Khatibi Wafa
Belkouchi Lina
El Haddad Siham
Allali Nazik
Chat Latifa
author_facet Oukassem siham
Abourak Chaimae
Khatibi Wafa
Belkouchi Lina
El Haddad Siham
Allali Nazik
Chat Latifa
author_sort Oukassem siham
collection DOAJ
description Embryonal tumors with multilayered rosettes (ETMR) are rare, highly aggressive brain neoplasms predominantly affecting children under 3 years of age. In the 2021 WHO Classification of Tumors of the Central Nervous System, ETMRs are classified as grade IV tumors, previously considered separate entities such as embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma (EBL). We report a 13-month-old girl who presented with afebrile seizures, left hemiparesis, and status epilepticus. Neuroimaging revealed a right fronto-parieto-temporal mass with typical features of ETMR, confirmed by stereotactic biopsy. Despite supportive care and preparation for surgery, the patient's condition deteriorated rapidly, and she passed away 15 days after admission. This case highlights the typical presentation, imaging characteristics, and poor prognosis of ETMR, emphasizing the importance of early recognition and molecular testing in enhancing diagnosis and treatment strategies
format Article
id doaj-art-8185fb684c724dd08dab95a55ee2b5cd
institution DOAJ
issn 1930-0433
language English
publishDate 2025-10-01
publisher Elsevier
record_format Article
series Radiology Case Reports
spelling doaj-art-8185fb684c724dd08dab95a55ee2b5cd2025-08-20T03:11:46ZengElsevierRadiology Case Reports1930-04332025-10-0120104890489410.1016/j.radcr.2025.06.042Rare and aggressive: A case of embryonal tumor with multilayered rosettes (ETMR) in a young childOukassem siham0Abourak Chaimae1Khatibi Wafa2Belkouchi Lina3El Haddad Siham4Allali Nazik5Chat Latifa6Corresponding author.; Department of Radiology, Mother-Child, Faculty of Medicine and Pharmacy of Rabat, Children's Hospital, Ibn Sina University Hospital, Mohammed V University, Rabat, MoroccoDepartment of Radiology, Mother-Child, Faculty of Medicine and Pharmacy of Rabat, Children's Hospital, Ibn Sina University Hospital, Mohammed V University, Rabat, MoroccoDepartment of Radiology, Mother-Child, Faculty of Medicine and Pharmacy of Rabat, Children's Hospital, Ibn Sina University Hospital, Mohammed V University, Rabat, MoroccoDepartment of Radiology, Mother-Child, Faculty of Medicine and Pharmacy of Rabat, Children's Hospital, Ibn Sina University Hospital, Mohammed V University, Rabat, MoroccoDepartment of Radiology, Mother-Child, Faculty of Medicine and Pharmacy of Rabat, Children's Hospital, Ibn Sina University Hospital, Mohammed V University, Rabat, MoroccoDepartment of Radiology, Mother-Child, Faculty of Medicine and Pharmacy of Rabat, Children's Hospital, Ibn Sina University Hospital, Mohammed V University, Rabat, MoroccoDepartment of Radiology, Mother-Child, Faculty of Medicine and Pharmacy of Rabat, Children's Hospital, Ibn Sina University Hospital, Mohammed V University, Rabat, MoroccoEmbryonal tumors with multilayered rosettes (ETMR) are rare, highly aggressive brain neoplasms predominantly affecting children under 3 years of age. In the 2021 WHO Classification of Tumors of the Central Nervous System, ETMRs are classified as grade IV tumors, previously considered separate entities such as embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma (EBL). We report a 13-month-old girl who presented with afebrile seizures, left hemiparesis, and status epilepticus. Neuroimaging revealed a right fronto-parieto-temporal mass with typical features of ETMR, confirmed by stereotactic biopsy. Despite supportive care and preparation for surgery, the patient's condition deteriorated rapidly, and she passed away 15 days after admission. This case highlights the typical presentation, imaging characteristics, and poor prognosis of ETMR, emphasizing the importance of early recognition and molecular testing in enhancing diagnosis and treatment strategieshttp://www.sciencedirect.com/science/article/pii/S1930043325005874ETMRMRIPediatric brain tumorPrognosis
spellingShingle Oukassem siham
Abourak Chaimae
Khatibi Wafa
Belkouchi Lina
El Haddad Siham
Allali Nazik
Chat Latifa
Rare and aggressive: A case of embryonal tumor with multilayered rosettes (ETMR) in a young child
Radiology Case Reports
ETMR
MRI
Pediatric brain tumor
Prognosis
title Rare and aggressive: A case of embryonal tumor with multilayered rosettes (ETMR) in a young child
title_full Rare and aggressive: A case of embryonal tumor with multilayered rosettes (ETMR) in a young child
title_fullStr Rare and aggressive: A case of embryonal tumor with multilayered rosettes (ETMR) in a young child
title_full_unstemmed Rare and aggressive: A case of embryonal tumor with multilayered rosettes (ETMR) in a young child
title_short Rare and aggressive: A case of embryonal tumor with multilayered rosettes (ETMR) in a young child
title_sort rare and aggressive a case of embryonal tumor with multilayered rosettes etmr in a young child
topic ETMR
MRI
Pediatric brain tumor
Prognosis
url http://www.sciencedirect.com/science/article/pii/S1930043325005874
work_keys_str_mv AT oukassemsiham rareandaggressiveacaseofembryonaltumorwithmultilayeredrosettesetmrinayoungchild
AT abourakchaimae rareandaggressiveacaseofembryonaltumorwithmultilayeredrosettesetmrinayoungchild
AT khatibiwafa rareandaggressiveacaseofembryonaltumorwithmultilayeredrosettesetmrinayoungchild
AT belkouchilina rareandaggressiveacaseofembryonaltumorwithmultilayeredrosettesetmrinayoungchild
AT elhaddadsiham rareandaggressiveacaseofembryonaltumorwithmultilayeredrosettesetmrinayoungchild
AT allalinazik rareandaggressiveacaseofembryonaltumorwithmultilayeredrosettesetmrinayoungchild
AT chatlatifa rareandaggressiveacaseofembryonaltumorwithmultilayeredrosettesetmrinayoungchild