Intracranial myxoid mesenchymal tumor with EWSR1 – CREB1 gene fusion: a case report and literature review

Abstract Intracranial myxoid mesenchymal tumors harboringEWSR1 fusions withCREB transcriptional factor gene families were recently described in several case reports and a few case series and this tumor closely resembles the myxoid variant of angiomatoid fibrous histiocytoma. We herein present an intracranial mesenchymal myxoid tumor arising in the third ventricle of a middle-aged woman. The tumor displayed prominent myxoid features consisting of mildly atypical oval to round cells, arranged in reticular and cord-like structures, with starburst-like amianthoid fibers, whereas it lacked pseudoangiomatoid spaces, pseudocapsules and lymphoid cuffing. Immunophenotypically, tumor cells were positive for EMA, desmin, and ALK (focal).EWSR1 andCREB1 rearrangements were identified using FISH assay. The proliferation index was low. It is currently uncertain whether these myxoid tumors represent a variant of angiomatoid fibrous histiocytoma or a novel tumor entity.
Source: Brain Tumor Pathology - Category: Neurology Source Type: research