Anatomy, physiology, and genetics of paragangliomas

Paragangliomas arise from an extra-adrenal paraganglionic cells, derived from the neural crest of the autonomic nervous system and make up the most common class of benign vascular neoplasms of the neck. PGLs of the head and neck originate most commonly from the paraganglia within the carotid body, the jugular foramen, the middle ear and the vagus nerve. Knowledge of the embryology, anatomy and genetics of these rare tumors is paramount to successful diagnosis and treatment of head and neck paragangliomas.