Ganglioneuroma of the Skull Base Presenting with Multiple Cranial Nerve Deficits: A Case Report and Review of the Literature

Objective: With this study, the authors aim to present a case of skull base ganglioneuroma presenting with cranial nerve deficits and to review the current literature regarding head and neck ganglioneuromas.

Methods and Results: A 60-year-old gentleman was admitted to an academic institution for sudden onset of neck pain, hoarseness, and dysphagia. He had deficits of both cranial nerves X and XII at presentation. A gadolinium-enhanced MRI revealed a 5 by 1.4 cm fusiform-enhancing lesion enlarging the right jugular foramen extending along the upper cervical spine and along the posterior aspect of the carotid sheath. A CT-guided biopsy was nondiagnostic, and systemic evaluation including PET imaging was negative for a primary malignancy. The patient underwent serial imaging, and then, due to persistent symptoms, a combined transmastoid and transcervical approach to the jugular foramen with complete resection of the parapharyngeal space lesion located on the sympathetic chain. His postoperative course was uneventful with a return of swallowing ability but without reconstitution of his presenting cranial nerve functional loss. Histopathologic examination was consistent with a ganglioneuroma. His serial preoperative imaging studies and intraoperative photo documentation are presented.

Conclusions: Ganglioneuromas are benign neurogenic tumors arising from autonomic ganglia. They commonly occur within the posterior mediastinum, retroperitoneum; they rarely occur on the neck. There are a limited number of reports of parapharyngeal ganglioneuromas at the skull base in the English language literature, and none to our knowledge presenting with these cranial nerve deficits. It is important to consider tumors derived from the sympathetic nervous system when approaching parapharyngeal lesions.