Head and neck paragangliomas are rare, slow-growing neuroendocrine tumors, with malignancy defined by metastatic spread rather than histopathological features [1, 3, 8]. Vagal paragangliomas comprise less than 5% of these tumors but have a higher risk of malignancy [3, 4].
Imaging with CT and MRI, including angiography, is essential for diagnosis and surgical planning, with the characteristic “salt and pepper” MRI appearance aiding differentiation from other lesions such as schwannomas [9, 10]. Recent advances in functional imaging, such as 68 Ga-DOTATATE PET/CT, have improved detection of multifocal and metastatic disease, especially in SDHx mutation carriers [12].
Recent multicenter registry data confirm that most head and neck paragangliomas are localized and slow-growing, with high long-term survival, even in metastatic cases [5]. Genetic testing is now widely recommended, as up to 50% of patients harbor pathogenic SDHx variants, which correlate with increased risk of multifocality and malignancy [5, 11]. The identification of SDHB mutations, in particular, is associated with a higher metastatic risk and warrants more intensive surveillance [12, 17].
The differential diagnosis of a poststyloid parapharyngeal mass involving the vagus nerve primarily includes vagal schwannomas, carotid body paragangliomas, and other rare parapharyngeal tumors. Vagal schwannomas, although neurogenic similar to paragangliomas, tend to grow more slowly and are less vascular, lacking the characteristic “salt and pepper” appearance on T2-weighted MRI seen in paragangliomas. Carotid body paragangliomas typically localize at the carotid bifurcation and displace the internal and external carotid arteries in a distinctive manner, which helps differentiate them from vagal paragangliomas. Other entities such as mucoepidermoid carcinomas or metastatic lymphadenopathy should be considered depending on clinical and radiological context. Multimodal imaging—including MRI, angiography, and functional imaging with 68 Ga-DOTATATE PET/CT—combined with histopathological analysis is essential to establish the diagnosis and guide management [11,12,13].
Surgical resection remains the mainstay of treatment but carries significant risk of cranial nerve morbidity owing to the tumor’s proximity to critical neurovascular structures [3, 8]. Recent surgical series emphasize the importance of early vascular control, intraoperative neuromonitoring, and the use of the operating microscope to optimize cranial nerve preservation and minimize blood loss [14, 16]. Preoperative embolization may be considered for large or highly vascular tumors to further reduce intraoperative bleeding, though its benefit remains debated [15].
Adjuvant radiotherapy, including stereotactic radiosurgery (SRS), is increasingly employed to improve local control and preserve function, especially in unresectable or residual tumors [6, 7, 13]. Recent studies report excellent tumor control and cranial nerve preservation with Gamma Knife SRS, achieving tumor volume reduction in up to 70% of cases and minimal neurological complications over a median follow-up of 25 months [13].
Chemotherapy is reserved for metastatic or progressive disease, though evidence remains limited. Newer targeted therapies and peptide receptor radionuclide therapy (PRRT) are emerging as potential options for advanced cases, particularly in patients with SDHB mutations or refractory disease [18, 19].
Optimal management of malignant vagal paraganglioma requires a multidisciplinary team, including otolaryngologists, radiologists, geneticists, radiation oncologists, and medical oncologists, to tailor treatment on the basis of tumor size, location, genetic status, and patient comorbidities [12, 13, 19].
