Paraganglioma that develops in the paraganglia other than adrenal medullar(extra-adrenal pheochromocytoma) is a relatively rare tumor that may be developed at any site where chromaffin tissue is existed. Such lesions have been estimated that as
many as
10% of retroperitoneal paragangliomas arise otside the adrenal gland and found in the organ of Zuckerkandl, urinary bladder, posterior mediastinum, skull base, carotid body, vagus nerve, glomus jugulare, larynx and small bowels. The organ of
Zuckerkandl
comprises a retroperitoneal cluster of paraganglia, that usually involutes during infancy, which is located along the abdominal aorta down to its bifurcation. Paraganglioma arising from this structure may become large enough to press the adjacent
structures and they are quite vascular and may be difficult to resect. The intravagal paraganglioma forms a palpable mass in the neck, closely surrounding or enveloping the vagus nerve. Chromaffin-reacting pheochromocytomas of the adrenal medulla
are
the most frequently encountered functional paraganglionic neoplasm. However extra-adrenal peochromocytomas as well as nonchromaffin-reacting paragangliomas may produce symptoms from catecholamine secretion. Three patients with functional and
non-functional paragangliomas of the organ of Zuckerkandl and non-functional intravagal paraganglioma of the right neck are reviewed together.
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