Lateral skull base paragangliomas (glomus tumors) are rare skull base tumors arising from neuroendocrine cells. These benign tumors can be locally aggressive with potential for intracranial extension and significant morbidity as they compromise cranial nerve structures. Treatment is highly patient dependent. Herein, we present a case of recurrent glomus vagale paraganglioma requiring a multidisciplinary transjugular and transcervical approach for complete resection.
A 64-year-old male was referred to the neurotology clinic in 2019 for a left skull base tumor causing progressive dysphonia and dysphagia. Exam revealed left true vocal fold weakness and no other abnormalities. Hearing was normal on the left. Magnetic resonance imaging (MRI) revealed a large hyperintense lesion of the left jugular foramen with intracranial cerebellopontine angle extension and normal flow through the sigmoid sinus and jugular vein. The patient elected for surgical removal and near-total resection was achieved via retrosigmoid craniotomy. A small portion was intentionally left in the jugular foramen to preserve the intact eleventh cranial nerve, internal jugular vein, and sigmoid sinus. Surgical pathology confirmed glomus paraganglioma. Postoperative radiation was strongly recommended, but the patient was lost to follow up due to the Covid-19 pandemic.
The patient re-presented in late 2021 with worsened dysphonia and dysphagia. Exam confirmed left true vocal fold immobility consistent with vagal nerve paralysis and a new finding of left tongue weakness consistent with hypoglossal nerve injury. MRI revealed recurrence of the lesion to dimensions larger than original presentation and complete occlusion of the sigmoid-jugular system. Hearing and facial nerve function remained fully intact, thus a transjugular approach with hearing preservation and complete surgical resection was utilized.
After combined retrosigmoid and transcervical incision, the transjugular approach was utilized to resect the sigmoid sinus, the tumor of the jugular foramen, and the intracranial extension. The ear canal and facial nerve canal were preserved. The sigmoid sinus was ligated with surgical clips and the jugular vein was ligated with suture thread. Intracranially, the hypoglossal nerve was identified and preserved, and the vagus nerve was seen eroded by tumor. Pathology confirmed recurrent paraganglioma. Postoperatively, the patient recovered well but continues to endorse persistent dysphonia. His treatment plan includes radiation and thyroplasty.
Multiple surgical approaches for the treatment of skull base paragangliomas have been reported including infratemporal types A to D, among others. This report identifies a rare case of recurrent paraganglioma which necessitated removal via transjugular approach. While uncommon in skull base surgery, this approach allowed identification and preservation of important neck and skull base structures (e.g., facial nerve, ear canal, spinal accessory nerve) while achieving complete gross resection. Radiation techniques have become popular alternatives for treatment of glomus tumors of the skull base due to high levels of surgery-related adverse events. Thus, skull base surgeons should be aware of the utility of the transjugular surgical technique for patients with intact hearing and facial nerve function who seek removal of intracranial jugular foramen tumors.