Paragangliomas Throughout the Body: Hereditary Syndromes and Imaging Features Including 3D Cinematic Rendering

Paragangliomas are highly vascular neuroendocrine tumors that arise from embryonic neural crest cells. They may either be hormonally active (sympathetic) or silent (parasympathetic). Approximately one-third are hereditary, and patients may develop multiple paragangliomas. Presurgical planning involves medical management as well as interventional and/or surgical techniques that must address vascular supply. Contrast-enhanced CT is the best initial anatomical study and shows the hypervascularity and feeding vessels. 3D cinematic rendering using multiplanar light sources can highlight spatial relationships of a mass to adjacent structures and help in surgical planning. This article will review the clinical presentation of paragangliomas, genetic syndromes, presurgical management, and tumor behavior and imaging appearance by location with a particular emphasis on 3D cinematic rendering.