Intracranial myxoid mesenchymal tumor with EWSR1-CREB1 fusion

Sasha Libbrecht¹, Joni Van Der Meulen², Veerle Mondelaers³, Edward Baert⁴, Caroline Vande Walle⁵, Jo Van Dorpe⁶, David Creytens⁷

Affiliations

¹ Department of Pathology, Ghent University Hospital, Ghent University, Ghent, Belgium.
² Center for Medical Genetics, Ghent University Hospital, Ghent University, Ghent, Belgium; CRIG, Cancer Research Institute Ghent, Ghent University Hospital, Ghent University, Ghent, Belgium.
³ CRIG, Cancer Research Institute Ghent, Ghent University Hospital, Ghent University, Ghent, Belgium; Department of Pediatric Hematology-Oncology and Stem Cell Transplantation, Ghent University Hospital, Ghent University, Ghent, Belgium.
⁴ Department of Neurosurgery, Ghent University Hospital, Ghent University, Ghent, Belgium.
⁵ Department of Radiology, Ghent University Hospital, Ghent University, Ghent, Belgium.
⁶ Department of Pathology, Ghent University Hospital, Ghent University, Ghent, Belgium; CRIG, Cancer Research Institute Ghent, Ghent University Hospital, Ghent University, Ghent, Belgium.
⁷ Department of Pathology, Ghent University Hospital, Ghent University, Ghent, Belgium; CRIG, Cancer Research Institute Ghent, Ghent University Hospital, Ghent University, Ghent, Belgium. Electronic address: david.creytens@uzgent.be.

PMID: 33080563
DOI: 10.1016/j.prp.2020.153239

Case Reports

Intracranial myxoid mesenchymal tumor with EWSR1-CREB1 fusion

Sasha Libbrecht et al. Pathol Res Pract. 2020 Dec.

. 2020 Dec;216(12):153239.

doi: 10.1016/j.prp.2020.153239. Epub 2020 Oct 2.

Authors

Sasha Libbrecht¹, Joni Van Der Meulen², Veerle Mondelaers³, Edward Baert⁴, Caroline Vande Walle⁵, Jo Van Dorpe⁶, David Creytens⁷

Affiliations

¹ Department of Pathology, Ghent University Hospital, Ghent University, Ghent, Belgium.
² Center for Medical Genetics, Ghent University Hospital, Ghent University, Ghent, Belgium; CRIG, Cancer Research Institute Ghent, Ghent University Hospital, Ghent University, Ghent, Belgium.
³ CRIG, Cancer Research Institute Ghent, Ghent University Hospital, Ghent University, Ghent, Belgium; Department of Pediatric Hematology-Oncology and Stem Cell Transplantation, Ghent University Hospital, Ghent University, Ghent, Belgium.
⁴ Department of Neurosurgery, Ghent University Hospital, Ghent University, Ghent, Belgium.
⁵ Department of Radiology, Ghent University Hospital, Ghent University, Ghent, Belgium.
⁶ Department of Pathology, Ghent University Hospital, Ghent University, Ghent, Belgium; CRIG, Cancer Research Institute Ghent, Ghent University Hospital, Ghent University, Ghent, Belgium.
⁷ Department of Pathology, Ghent University Hospital, Ghent University, Ghent, Belgium; CRIG, Cancer Research Institute Ghent, Ghent University Hospital, Ghent University, Ghent, Belgium. Electronic address: david.creytens@uzgent.be.

PMID: 33080563
DOI: 10.1016/j.prp.2020.153239

Abstract

Myxoid mesenchymal tumor with predilection for intracranial location harboring EWSR1 fusions with CREB family transcription factors is a recently described and exceedingly rare neoplasm. While some debate still exists whether this is a true separate entity or a myxoid variant of angiomatoid fibrous histiocytoma, these tumors still deserve separate attention due to localization, fairly distinct histology and higher incidence in the pediatric population. Data regarding outcome of these neoplasms are still sparse in medical literature. We report a case of an intracranial myxoid tumor with EWSR1-CREB1 fusion in a 14-year-old girl.

Keywords: Angiomatoid fibrous histiocytoma; CREB; EWSR1; Fluorescence in situ hybridization; Intracranial myxoid mesenchymal tumor.

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Intracranial myxoid mesenchymal tumor with EWSR1-CREB1 fusion

Affiliations

Intracranial myxoid mesenchymal tumor with EWSR1-CREB1 fusion

Authors

Affiliations

Abstract

Publication types

MeSH terms

Substances

Supplementary concepts

LinkOut - more resources

Full Text Sources

Medical