USF-LVHN SELECT
Adult Intracranial Myxoid Mesenchymal Tumor with EWSR1-ATF1 Gene Fusion.
Publication/Presentation Date
11-1-2020
Abstract
BACKGROUND: Intracranial myxoid mesenchymal tumors (IMMTs) carrying an EWSR1-CREB gene family fusion are extremely rare and have only been identified in 10 previous reports. All but one has been found in children or young adults. Although there appear to be similarities to a myxoid variant of angiomatoid fibrous histiocytoma (AFH), clear histologic differences exist that appear to distinguish IMMTs as a distinct and novel entity. Previous reports have lacked detailed long-term follow-up and recommendations regarding treatment approach.
CASE DESCRIPTION: This case describes a 48-year-old woman who presented with a left intraventricular mass that was identified on histology as an IMMT with an EWSR1-ATF1 gene fusion. After initial resection, the tumor demonstrated local recurrence. Repeat resection was performed followed by immediate demonstration of local and distant tumor recurrence. Histologic analysis of the tumor demonstrated a myxoid mesenchymal tumor distinct from AFH. Fractionated stereotactic radiation therapy was administered after the second resection, and tumor control was achieved at 1 year.
CONCLUSIONS: Intracranial myxoid mesenchymal tumor is a novel and rare entity that has been previously identified in only 10 cases. This case is particularly remarkable because it is only the second IMMT case to occur in a middle-aged adult and shares striking similarities in clinical presentation to the previously reported case. Given the aggressive recurrence seen with the presented case, we recommend the treatment plan to be surgical resection followed by adjuvant radiation therapy.
Volume
143
First Page
91
Last Page
96
ISSN
1878-8769
Published In/Presented At
Ward, B., Wang, C. P., Macaulay, R. J. B., & Liu, J. K. C. (2020). Adult Intracranial Myxoid Mesenchymal Tumor with EWSR1-ATF1 Gene Fusion. World neurosurgery, 143, 91–96. https://doi.org/10.1016/j.wneu.2020.07.057
Disciplines
Medical Education | Medicine and Health Sciences
PubMedID
32683005
Department(s)
USF-LVHN SELECT Program, USF-LVHN SELECT Program Students
Document Type
Article