DOI: https://doi.org/10.36347/sjmcr.2025.v13i10.048

Pages: 2397-2401

Chondroid chordoma is a rare skull-base tumor of notochordal origin that radiologically mimics chondrosarcoma, making histopathology and immunohistochemistry essential for diagnosis. We report a 22-year-old man with progressive occipital headache, imbalance, diplopia, and tinnitus. CT and MRI revealed a destructive midline retroclival mass encasing the basilar and cavernous carotid arteries with brainstem compression. A transmaxillary–transclival approach achieved gross total resection. Histology showed physaliphorous cells within a myxoid/chondroid matrix. Immunohistochemistry demonstrated EMA, S100, and vimentin positivity with preserved INI-1 and low Ki-67 (1–2%), confirming chondroid chordoma. Postoperative MRI identified small residual tumor at the left petrous apex and cavernous sinus. The patient recovered to WHO performance status 0 except for persistent diplopia and was referred for adjuvant high-dose intensity-modulated radiotherapy (IMRT). This case illustrates the diagnostic challenge of clival chondroid chordoma, the limits of safe resection in the skull base, and the need for multidisciplinary management with high-dose conformal radiotherapy. Although historically thought less aggressive, chondroid chordoma shares the recurrence risk and long-term prognosis of conventional chordoma, warranting lifelong imaging surveillance