A Rare Case of Bilateral Chondrosarcoma of the Jugular Foramen: Management Strategy, Surgical Approach and Long-Term Outcomes

Background: Chondrosarcomas (CS) are slow-growing malignant cartilaginous tumors. They are extremely rare (about 0.15% of head and neck tumors) and poorly aggressive. They generally occur as single lesions in the skull base, but as shown in our case report, they can be bilateral. G1 CS is known to be poorly radiosensitive. The gold standard therapy for G1 CS is radical surgical removal. Methods: Our case report refers to a bilateral jugular foramen (JF) CS in a 22-year-old woman presenting with right diplopia caused by a VI nerve paralysis. MRI was performed that showed a bilateral lesion invading the petro-occipital junction and the JF. A transmastoid biopsy was performed and histology showed chondrosarcoma G1. The bilateral involvement of the JF was a demanding problem to manage, both in the treatment strategy and in the surgical approach. As surgery would involve the closure of sigmoid sinus and the jugular bulb complex bilaterally, the patient was approached with a staged surgery (petro-occipital transigmoid approach), after consecutive angiographic studies to assess the patency of the collateral venous network. Radical excision was achieved bilaterally, no cranial nerve deficit was recorded in the postoperative period, hearing and facial nerve were normal. Conclusions: The patient till date is alive without disease, with a bilaterally closed jugular foramen complex and no postoperative sequelae. MRI shows no recurrence of disease at 12 years of follow-up.