Mesenchymal chondrosarcoma of the brain is one of the rarest tumors with dismal prognosis. A 26-year-old man presented with headache, vomiting, and diplopia. On evaluation, a moderately enhancing extra-axial lesion was seen in right temporal region involving right greater wing of sphenoid, adjacent right maxillary sinus, and lateral wall of right orbit. Patient underwent magnetic resonance imaging of the spine and computed tomography (CT) of the chest for possible metastasis which showed lesion in lumbar vertebrae and left lung. Patient was planned for CT-guided lung biopsy which proved inconclusive. The patient was further planned for craniotomy and underwent craniotomy with microscopic excision of the mass. Histoimmunochemistry was suggestive of extraskeletal mesenchymal chondrosarcoma. Mesenchymal chondrosarcoma of the brain is highly aggressive tumors which are difficult to differentiate radiologically. Radical excision followed by chemoradiotherapy is optimal treatment of choice.
Keywords: extraskeletal mesenchymal chondrosarcoma; intracranial tumor; metastasis.