Orbital chordoid meningioma: a rare entity with an aggressive clinical course

Chordoid meningioma is a rare histological variant of meningioma classified as World Health Organization Grade II because of its aggressive clinical behavior and high recurrence potential. Orbital involvement is exceedingly uncommon and poses significant diagnostic and therapeutic challenges. We report a long-term clinical course of a patient with orbital chordoid meningioma who initially presented with progressive proptosis and visual impairment. Despite multiple biopsies, radiotherapy, and long-term follow-up, the tumor demonstrated locally aggressive behavior with repeated progression, ultimately requiring orbital exenteration. Histopathological examination confirmed chordoid meningioma with a positive reactivity against SSTR2, and progressively increasing Ki-67 proliferation index, supporting its aggressive nature. This case highlights the diagnostic difficulties, limited response to conventional treatment modalities, and the importance of long-term surveillance in orbital chordoid meningioma.