Extraskeletal myxoid chondrosarcoma of the jugular foramen

Objective: To report a case of an extraskeletal myxoid chondrosarcoma (EMC) arising from the jugular foramen. EMCs are tumors usually seen in the deep soft tissues of the extremities and are rarely seen within the intracranial cavity. The histological differential diagnosis includes chordoma, conventional chondrosarcoma and chordoid meningioma, among others. A distinguishing feature of EMC is their characteristic reciprocal translocation t(9;22)(q22;q12). Material: A 63-year-old man presented with progressive hearing loss and gait imbalance. Magnetic resonance imaging showed a heterogeneously enhancing 2.4 cm mass in the cerebellopontine angle. A right far lateral transcondylar skull base approach with gross total removal of the tumor was performed. Intraoperative findings showed that the mass appeared to arise from the glossopharyngeal nerve within the jugular foramen. Method: Histology, immunohistochemistry, and fluorescence in situ hybridization studies were performed. Results: Histological and immunohistochemical studies were compatible with the diagnosis of EMC. Fluorescence in situ hybridization studies showed disruption of the EWS gene locus at 22q12 and added further support to the diagnosis. Conclusions: We report a rare case of EMC arising from the jugular foramen, and the diagnosis of EMC can be supported by confirmation of disruption of the EWS gene locus.