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Orbito-cranial mesenchymal chondrosarcoma in a young female: A rare case report

Mesenchymal chondrosarcoma (MCS) of the head and neck is highly aggressive malignant small round cell neoplasm with cartilaginous differentiation, often with a pericytomatous vascular pattern. It represents approximately 0.1% of all head and neck neoplasms. Chondrosarcoma of orbit is extremely rare. We report a case of orbito-cranial MCS in a 24-year-old female who presented with exophthalmos. Imaging revealed a large heterogenous lesion arising from right orbit with intracranial extension. Patient underwent right fronto-temporal craniotomy with tumor decompression from intracranial part and right orbit. Histopathologic examination and immunohistochemistry was suggestive of MCS. Postoperative residual disease was treated with chemotherapy showing partial response.