Non-C19MC-altered embryonal tumor with multilayered rosettes in a young woman with DICER1 syndrome: case report and review of the literature.
Pathologica
; 116(3): 170-175, 2024 Jun.
Article
in En
| MEDLINE
| ID: mdl-38979591
ABSTRACT
Embryonal tumors with multilayered rosettes (ETMR) are highly aggressive and therapy-resistant pediatric central nervous system (CNS) tumors that have three histological patters embryonal tumor with abundant neuropil and true rosettes, ependymoblastoma, and medulloepithelioma. We present a case of ETMR in an 18-year-old woman with DICER1 syndrome. This report confirms the important role of DNA-methylation analysis in the classification of CNS embryonal tumors and the importance of investigating somatic and germline DICER1 mutations in all CNS embryonal tumors.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Neoplasms, Germ Cell and Embryonal
/
Ribonuclease III
/
DEAD-box RNA Helicases
Limits:
Adolescent
/
Female
/
Humans
Language:
En
Journal:
Pathologica
Year:
2024
Type:
Article
Affiliation country:
Spain