[First description of mesenchymal chondrosarcoma of the lateral skull base in a 9-year-old boy]

Mesenchymal chondrosarcoma (MSC) is a very rare sarcoma that is highly malignant with frequent local recurrences and distant metastases leading to a very poor long-term outcome. It differs from classical chondrosarcomas in that it occurs more often in children and commonly in extraskeletal locations. Hardly any evidence-based recommendations for treatment exist. We present the case of a 9-year old boy (pre- and intraoperative findings and histopathological details) who was initially admitted with the diagnosis of a jugular paraganglioma and discuss this case in the light of the recent literature.