Chordoid Meningioma Along the Sphenoid Wing: A Case Report and Brief Review of the Literature

Chordoid meningiomas (CMs) are rare WHO grade 2 CNS meningiomas that exhibit aggressive behavior and a high rate of recurrence. Diagnosis of CMs can be challenging, as they closely mimic other distinct tumors, such as true chordomas, chondrosarcomas, or chordoid gliomas. An accurate diagnosis requires a careful combination of MRI characteristics, immunohistochemistry (IHC) analysis, and review by an experienced neuropathologist. 
We report a 49-year-old patient with a six-month history of progressive frontal headaches, intermittent blurred vision in the right eye, and dysphasia. Examination revealed subtle papilledema, mild anomic aphasia, and a right superior quadrantanopia. Brain magnetic resonance imaging (MRI) showed a heterogeneous 4.2 × 3.8 cm extra-axial mass near the left sphenoid wing with broad dural attachment, homogeneous enhancement, and marked peritumoral edema.
Because of proximity to the cavernous sinus, the patient underwent subtotal tumor resection (Simpson grade IV). Histologic examination showed cords of epithelial tumor cells in a highly cellular myxoid stroma, with frequent mitoses and focal brain invasion. IHC was positive for SSTR2A (somatostatin receptor) and EMA and negative for brachyury, supporting CM and excluding chordoma. Molecular testing identified an NF2 mutation and a DNA methylation profile consistent with CM. The patient received intensity-modulated radiation therapy after surgery. A local recurrence developed at 26 months and was treated with stereotactic radiosurgery. At 36-month follow-up, the tumor size remained stable. This case highlights the diagnostic challenges, differential diagnosis, and management of this rare tumor and includes a brief review of the literature.