Paragangliomas are rare hypervascular neuroendocrine tumors that originate from paraganglion cells outside the adrenal medulla, contrary to pheochromocytomas which originate from paraganglion cells within it [1,2]. Extra-adrenal paraganglion cells allow, between other functions, body response to stresses such as hypoglycemia, hypercapnia, and hypoxia [3].

In the head and neck region, paragangliomas present in characteristic locations which define their name, most commonly in the carotid bifurcation, along the vagus nerve, in the middle ear and jugular foramen (or both) [3]. Less common locations include the larynx, paranasal sinuses, and the pterygopalatine fossa, among others [1]. In the head and neck region, it most commonly affects females, and patients in the 4^th decade [2]. Most are benign, but may be intimately related to surrounding structures. Up to 13% are malignant, defined by the appearance of regional adenopathies, or uncommonly distant metastasis. Most cases are sporadic, while about 40% are hereditary, possibly associated with certain syndromes, such as von Hippel-Lindau disease, neurofibromatosis type I, and multiple endocrine neoplasia type II [3]. They are also associated with mutations of the germline succinate dehydrogenase (SDH) subunits D and B, and in such patients there is an increased risk for multifocal primary disease and metastatic disease, respectively. The most common clinical presentation is in the form of a slow growing painless mass, although it can vary according to the location of the lesion [1].