Suprasellar masquerader: Chordoid glioma

Background: Chordoid glioma is a rare well-circumscribed glial neoplasm arising in adults and predominantly affects females. Tanycytes of the third ventricle have been proposed as the cell of origin owing to its location. It is characterized by chordoid features with myxoid and inflammatory stroma and recurrent PRKCA p.D463H mis-sense mutation. Case report: We present two cases (30-year-old female and 45-year-old male) with similar complaints of behavioral change and headache. Midline suprasellar homogeneously enhancing mass was seen on contrast-enhanced magnetic resonance imaging. Histopathology and immunohis-tochemistry was characteristic of chordoid glioma with cords and clusters of epitheli-oid cells arranged in a solid pattern. There were variable amounts of myxoid stroma and lymphoplasmacytic infiltrate. No mitosis, ne-crosis, or brain invasion was noted. The cells expressed strong diffuse positivity for glial fi-brillary acid protein (GFAP) and weak nuclear thyroid transcription factor (TTF-1). Epithe-lial membrane antigen (EMA)and brachyury were negative. Subsequently, the lady under-went gross total excision and died soon after the operation. The male patient received ra-diotherapy and is currently doing well after 6 months of follow-up. Conclusion: The rare occurrence as well as the radiological and morphological overlaps in chordoid gliomas make them a true masquerader. Combina-tion of GFAP and TTF-1 in the immunohisto-chemical panel can be useful in differential diagnosis. Mainstay of treatment is complete surgical excision, with adjuvant radiotherapy becoming increasingly important.