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KMID : 0371320010610060619
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Journal of the Korean Surgical Society
2001 Volume.61 No. 6 p.619 ~ p.624
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Paraganglioma Arising from Retroperitoneum, Mediastinum
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In Seung-Hyun
Kwon Oh-Jung
Park Jae-Jeong
Jung Pa-Jong
Lee Heung-Woo
Kwak Jin-Young
Chung Won-Sang
Lee Kwong-Soo
Lee Kyeong-Geun
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Abstract
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A paraganglioma that arises from the Retroperitoneum, mediastinum is an extra-adrenal form of pheochromcytoma. Extra-adrenal pheochromocytomas can occur at any site in the abdomen where chromaffin tissue is located and have been found in the
paravertebral ganglia, the organ of Zuckerkandl, and the urinary bladder. An extra-adrenal pheochromocytomas can arise from a collection of para-aortic, paraganglion cells located at the origin of the inferior mesenteric artery, This collection
of
paraganglia was first described in fetuses by Zuckerkandl in 1901 and has subsequently been referred to as the organ of Zuckerkandl. Extra-adrenal pheochromocytoma generally secrete norepinephrine, not epinephrine, because there is an absence of
the
enzyme phenyl- ethanolamine-N-methyl transferase. Therefore symptoms are related to the production of norepinephrine. Extra-adrenal pheochromocytomas pursue a more aggressive course than their adrenal counterparts. It was found to metastasize in
approximately 20% to 40% of cases, compared with only a 2% to 10% rate of metastasis in all adrenal tumors. We report on a three case of paragangliomas that had a functional and non-functional paraganglioma of the organ of Zuckerkandl and a case
of
a
non-functional paraganglioma of the posterior mediastinum.
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