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A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites (including the head, neck, thorax and abdomen). About 97% are benign and cured by surgical removal; the remaining 3% are malignant because they are able to produce distant metastases. Paragangliomas are still sometimes referred to using older, obsolete terminology (for example as "chemodectomas" or "glomus jugularis", the latter not to be confused with glomus tumors of the skin).

Cellular origin and classification

Paragangliomas originate from chromaffin cells in paraganglia or chromaffin-negative glomus cells derived from the embryonic neural crest, functioning as part of the sympathetic nervous system (a branch of the autonomic nervous system). These cells normally act as special chemoreceptors located along blood vessels, particularly in the carotid bodies (at the bifurcation of the common carotid artery in the neck) and in aortic bodies (near the aortic arch).

Accordingly, paragangliomas are categorised as originating from a neural cell line in the World Health Organization classification of neuroendocrine tumors. In the categorization proposed by Wick, paragangliomas belong to group II. Given the fact that they originate from cells of the orthosympathetic system, paragangliomas are closely related to phaeochromocytomas, which however are chromaffine-positive.

Clinical presentation

Most paragangliomas are...
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