Intracranial extraskeletal Myxoid Chondrosarcoma in CNs-An exceptionally Rare Case Report

Extraskeletal myxoid chondrosarcoma is a rare soft tissue sarcoma. Though usually indolent it can have aggressive course. Since its recognition as a distinct clinicopathologic entity, now considered an intermediate grade neoplasm rather than a low grade neoplasm as previously considered. They are most commonly located in the extremities, limb girdles or trunk. However, unusual locations have also been reported including central nervous system, nasal cavity breast and vulva. In CNS there have been very few cases (less than 10) of EMC reported predominantly in the intracranial location with sites of origin including the choroid plexus, pineal gland and cortex.

We are reporting a case of 35 years female with complaints of headache, severe bilateral proptosis with loss of vision and altered sensorium. MRI revealed a predominantly cystic mass lesion involving the anterior skull base, paranasal sinus and nasal cavity. Intraoperative crush cytology diagnosed it as an extra skeletal mesenchymal tumor with chondrocytic differentiation which was confirmed on histopathology and marker studies as extra skeletal myxoid chondrosarcoma.