Paraganglioma: Difference between revisions
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=== Epidemiology === | === Epidemiology === | ||
The incidence of head and neck paragangliomas has been reported as 1 in 30,000 to 1 in 100,000. Approximately 10% of patients with head and neck paragangliomas will have multiple masses. As many as 25-35% of cases have been linked to hereditary conditions, typically Familial Paraganglioma Syndrome (see Genetics section below). There is an equal distribution of carotid paragangliomas in men and women, but jugulotympanic paragangliomas are six times more likely to be in women. | The incidence of head and neck paragangliomas has been reported as 1 in 30,000 to 1 in 100,000. Approximately 10% of patients with head and neck paragangliomas will have multiple masses. As many as 25-35% of cases have been linked to hereditary conditions, typically Familial Paraganglioma Syndrome (see [[Paraganglioma#Genetics|Genetics]] section below). There is an equal distribution of carotid paragangliomas in men and women, but jugulotympanic paragangliomas are six times more likely to be in women. | ||
=== Genetics === | === Genetics === | ||
Revision as of 13:37, 7 September 2024
Overview
Paragangliomas are masses derived from the paraganglia, a group of non-neuronal cells that are associated with the sympathetic and parasympathetic nervous system.
History
These tumors historically were called "glomus tumors" when present in the head and neck. This term has fallen out of use due to potential confusion with other structures with similar names, such as glomus bodies.
Pathophysiology
Relevant Anatomy
The term "paraganglia" refers to a group of non-neuronal neuroendocrine cells derived embryologically from neural crest cells. There are paraganglia associated with the sympathetic nervous system comprised of chromaffin cells, and those associated with the parasympathetic nervous system comprised of glomus cells. The adrenal medulla is the largest collection of Chromaffin cells in the body. The paraganglia of the head and neck region are associated with the glomus cells of the parasympathetic nervous system.
The paraganglia are highly vascularized for the purposes of chemoreceptor sensitivity. Sympathetic paraganglia act as endocrine organs with systemic catecholamine release (such as the adrenal medulla, organ of Zuckerkandl, etc.). These paraganglia are a major source of catecholamines in early embryogenesis (assuming much function of the adrenal medulla). Parasympathetic paraganglia predominantly have more local effects on nerve endings, such as in the carotid body.
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Target end-organs of the sympathetic nervous system.
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Target end-organs of the parasympathetic nervous system.
Disease Etiology
Epidemiology
The incidence of head and neck paragangliomas has been reported as 1 in 30,000 to 1 in 100,000. Approximately 10% of patients with head and neck paragangliomas will have multiple masses. As many as 25-35% of cases have been linked to hereditary conditions, typically Familial Paraganglioma Syndrome (see Genetics section below). There is an equal distribution of carotid paragangliomas in men and women, but jugulotympanic paragangliomas are six times more likely to be in women.
Genetics
Histology
Diagnosis
Patient History
Most patients present for evaluation in their 40s or 50s.