Treatment of Head and Neck Paragangliomas

Kenneth Hu, MD; Mark S. Persky, MD


Cancer Control. 2016;23(3):228-241. 

In This Article


Paragangliomas are rarely malignant, representing a small subset of extra-adrenal paragangliomas with a propensity for regional lymph-node and distant metastatic disease, primarily to bone, the lungs, and liver.[1–3] A higher rate of malignancy has been observed in sporadic paragangliomas compared with the familial type, with the exception of the familial syndrome associated with SDHB mutation (rate of malignancy, 30%–70%).[10,11] The rate of malignancy depends on the site of origin: Although rare, orbital and laryngeal paragangliomas have a 25% rate of malignancy, which is the highest rate of any paraganglioma.[1–3] By comparison, the rate of malignancy for vagal paragangliomas is between 16% and 19%, between 5% and 6% for jugulotympanic paragangliomas, and between 3% and 4% for carotid body tumors.[10] No histological criteria exist to diagnose malignancy in primary tumors. The health care professional can make the diagnosis only if malignancy is confirmed by the presence of a tumor in the lymph nodes or the disease has metastatically spread to distant sites.[1–3]

It is worth emphasizing that the implications for treatment are significant when malignant disease is present.[11–13] Locoregional control is best achieved with primary resection followed by adjuvant radiotherapy.[1–3] The disease is known to recur for up to 20 years.[1–3] The 5-year survival rates are 50% to 80% for those with nodal disease and up to 11% for those with distant spread.[14]