Intracranial myxoid mesenchymal tumor with EWSR1-CREB1 fusion

Pathol Res Pract. 2020 Dec;216(12):153239. doi: 10.1016/j.prp.2020.153239. Epub 2020 Oct 2.


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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Biomarkers, Tumor / genetics*
  • Brain Neoplasms / diagnostic imaging
  • Brain Neoplasms / genetics*
  • Brain Neoplasms / pathology
  • Diagnosis, Differential
  • Female
  • Gene Fusion*
  • Histiocytoma, Malignant Fibrous / diagnostic imaging
  • Histiocytoma, Malignant Fibrous / genetics*
  • Histiocytoma, Malignant Fibrous / pathology
  • Humans
  • In Situ Hybridization, Fluorescence
  • Oncogene Proteins, Fusion / genetics*
  • Predictive Value of Tests


  • Biomarkers, Tumor
  • EWSR1-CREB1 fusion protein, human
  • Oncogene Proteins, Fusion

Supplementary concepts

  • Histiocytoma, Angiomatoid Fibrous