Primary Chondrosarcoma of the Jugular Foramen: Approach Selection and Review of Literature

Skull base chondrosarcoma is exceedingly rare, representing only 0.2% of all intracranial tumors. The etiology of skull base chondrosarcoma is not well understood. Most common location is petroclival region with extension to the adjoining regions. These lesions most commonly present with compressive cranial neuropathies. We present a rare case of a 42 year old female with a chondrosarcoma which was primarily located in the left jugular foramen with involvement of hypoglossal canal and clivus. The patient presented with worsening hoarseness and dysphagia over several months and left vocal cord paralysis on flexible laryngoscopic exam. Weakness of cranial nerves IX, X, XI, and XII noted at time of presentation. CT and MRI revealed a lesion of the left jugular foramen, which was subsequently biopsied via a transnasal route with pathology findings compatible with that of chondrosarcoma. Representative pre-operative MR imaging of the tumor is demonstrated in [Figs. 1]–3.

Patient's case was presented at multidisciplinary skull base tumor board where surgical resection was recommended as the initial treatment with plans of adjuvant proton radiation treatment. Surgical access was performed via a transtemporal transjugular approach to the posterior cranial fossa and jugular foramen. Ultimately, a subtotal resection of the tumor was achieved which is shown in Fig. 4. Layered closure of the defect was performed including abdominal fat graft placement and layered ear canal closure.

Lumbar drain was placed and subsequently removed on post-op day 3 after no signs of CSF leak. Patient was discharged home on post-op day 4 after an uncomplicated hospital stay. Patient's baseline preoperative cranial nerve deficits remained unchanged with no new cranial nerve deficits observed. The diagnosis of chondrosarcoma was confirmed on final pathology results.

To our knowledge, there are no reports of primary jugular foramen location of chondrosarcoma. Carlson et al proposed a staging system for petroclival chondrosarcomas in a case series of 55 cases. Various surgical approaches have been proposed with subtemporal surgical approaches most commonly used. Of those patients who underwent subtotal resection, 64% of patients received adjuvant radiation therapy, which is planned for the patient in this case in the form of proton beam radiation. Ultimately, multimodal treatment reduces rate of recurrence and allows long-term excellent local control.

Fig. 1 (A) Pre-op T1 contrasted axial imaging shows a heterogenous mass in the left skull base centered in the jugular foramen with extension to the nasopharyngeal carotid space, hypoglossal canal, and clivus. The internal jugular vein and jugular bulb are involved with tumor. (B) Pre-op T2 axial imaging showing a right normal jugular foramen and left one involved with tumor. (C) Pre-op T2 axial imaging at level of hypoglossal canal showing expansion of hypoglossal canal with the tumor.

Fig. 2 Post-op T1 contrasted axial imaging showing subtotal resection of jugular foramen tumor.

Publication History

Article published online:
15 February 2022