Jugular Foramen Chondrosarcoma Masquerading as Intracranial Venous Thrombosis

Introduction: Chondrosarcomas (CSAs) are rare, slow growing, locally aggressive malignancies originating from multipotent mesenchymal cells or embryonic cartilaginous remnants. CSAs of the skull base are rare, accounting for 0.15% of head and neck neoplasms and 6% of skull base tumors. Eighteen CSAs arising from the jugular foramen (JF) have been reported in the English literature. Here, we present a unique case of CSA of the JF with intravascular invasion into the jugular venous system that was treated with a multidisciplinary surgical approach.

Materials and Methods: A case report and review of the literature.

Results: A 54-year-old male with well-controlled HIV presented with 1-year of dysphagia, dysphonia, imbalance, and pulsatile tinnitus. Examination revealed left uvular deviation, right tongue deviation, and right shoulder weakness. Imaging with CT and MRI revealed a right JF mass with concern for schwannoma or paraganglioma with occlusion of ipsilateral sigmoid sinus and patency on the contralateral side ([Fig. 1]). Angiogram showed right sigmoid-jugular bulb junction occlusion with collateralization of the venous drainage to the pterygoid plexus and cervical/muscular venous branches. He underwent a modified infratemporal and transcervical approach for excision with neurotology, neurosurgery, and head and neck surgical oncology. The tumor was infiltrating the mastoid, eroding the posterior fossa endolymphatic sac ([Fig. 2]), and posterior semicircular canal, invading the sigmoid sinus, and completely filling the jugular bulb and internal jugular vein. There was no venous outflow from the sigmoid sinus intravascularly due to complete occlusion proximally. Histopathology confirmed grade 2 CSA. The tumor was grossly resected and removed from the vasculature. Flow was reestablished after the tumor was removed from within the sigmoid sinus which was then extradurally packed for hemostasis. The posterior semicircular canal was plugged (Fig. 3). The patient’s hearing and facial function were preserved. His cranial nerve deficits recovered, including the resolution of dysphagia with clearance for regular diet after swallow therapy. He had post-op vertigo and nystagmus and underwent vestibular rehabilitation. He is being observed with the option of radiosurgery in case of recurrence. It is important to distinguish primary CSAs arising in the JF from those secondarily spread to the JF; the former of which is much rarer with limited data regarding treatment protocols and patient outcomes. Most patients present with various lower cranial nerve deficits, similar to our patient. Generally, CSAs primarily arising in the JF have a favorable prognosis where data are available. Nine (50%) patients were disease-free at their last follow-up, and another two (11.1%) patients demonstrated residual tumors on imaging without further growth. No JF CSAs reported in the literature have metastasized. One (5.5%) patient died secondary to pneumonia in the postoperative period, and one (5.5%) experienced recurrence five years after surgery and died secondary to a brainstem infarct.

Fig. 1

Fig. 2

Conclusion: CSAs primarily arising from the JF are rare malignancies with limited data on treatment protocols and outcomes. We described a case successfully managed with a multidisciplinary surgical approach, leading to gross total resection with no perioperative complications.

 

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No conflict of interest has been declared by the author(s).

Publication History

Article published online:
07 February 2025