Correlation Engine 2.0
Clear Search sequence regions

Sizes of these terms reflect their relevance to your search.

Myxoid mesenchymal tumours harbouring fusions between EWSR1 and the CREB family transcription factors have recently been described. Whether they represent a novel entity or a myxoid variant of angiomatoid fibrous histiocytoma (AFH) remains a matter of debate. We describe the case of a 58-year-old woman with a previous history of breast cancer that developed progressive neurological decline due to a large mass located in the left lateral ventricle of the brain. Histology revealed a mesenchymal tumour with multinodular growth, variable cellularity, prominent myxoid stroma and numerous amianthoid fibres. No evidence of pseudo-capsule or lymphoid cuffing was identified. RNA sequencing disclosed EWSR1-CREB1 gene fusion. Only 20 cases of intracranial mesenchymal tumours harbouring these translocations have been described, mostly in adolescents and young adults and with dural attachment. Occurrence in this age group and with intraventricular location has been even more rarely reported. A better understanding of tumour behaviour is needed to establish treatment guidelines and improve patient outcome.


Pedro Valente Aguiar, Jorge Pinheiro, Jorge Lima, Rui Vaz, Paulo Linhares. Myxoid mesenchymal intraventricular brain tumour with EWSR1-CREB1 gene fusion in an adult woman. Virchows Archiv : an international journal of pathology. 2021 May;478(5):1019-1024

Expand section icon Mesh Tags

Expand section icon Substances

PMID: 32632473

View Full Text