FIGURE 5.

Intracranial mesenchymal tumor with FET‐CREB fusion can histologically mimic chordoid meningioma. Patient ATF1 #2 is a 9‐year‐old female who presented with an extra‐axial mass along the cerebral convexity of the left frontal lobe. Surgical resection demonstrated a neoplasm composed of cords and clusters of epithelioid cells in a mucin‐rich stroma closely resembling chordoid meningioma. However, the tumor was negative for SSTR2A expression and contained small foci of tumor cells with desmin expression. Genomic interrogation revealed an in‐frame EWSR1‐ATF1 fusion and absence of mutations involving NF2 and other genes characteristic of meningioma