A male patient with age of 29, hypertension, headache, palpitation, sweating was detected the tumor in the left wall of the abdominal aorta. The tumor was removed by operation in Viet Duc hospital in 12th October 1999. The results of anatomy also found that the preoprative diagnosis of the paraganglioma, zuckerkandl tumor.
Paraganglioma ; Para-Aortic Bodies

No abstract available.
Para-Aortic Bodies* ; Paraganglioma*

Extra-adrenal abdominal paragangliomas are rare. Most arise from the organs of Zuckerkandl, involve large concentrations of paraganglionic tissue, and are located in the para-aortic space along the sympathetic chain. Published reports have, however, described normal paraganglionic tissue at the root of the mesentery which serves as the superior limit of the organs of Zuckerkandl, and mesenteric paraganglioma is very rare. We report a case of paraganglioma with cystic degeneration arising from the mesentery.
Mesentery ; Para-Aortic Bodies ; Paraganglioma*

PURPOSE: Retroperitoneal paraganglioma is a relatively uncommon neurogenic tumor, arising from paraganglial tissue. In our knowledge, there is few report about the radiologic findings of extraadrenal paraganglioma, therefore, here we document the adiologic findings of retroperitoneal paraganglioma with pathologic correlation. MATERIALS AND METHODS: In 5 patients with surgicopathologically confirmed extraadrenal paraganglioma and 1 clinjcolaboratorily confirmed case, we analyzed the ultrasonographic, computed tomographic and MRI findings, and correlated them with gross pathologic specimen. RESULTS: The location of the tumors was medial aspect of left kindey(n=2), superomedial aspect of right kidney hilum(n=2), and organ of Zuckerkandl area(n=2), UItrasonoram showed well-marginated mass(n=5), occasionally with irregular central necrosis with or without partially echogenic area suggesting hemorrhage(n=2). CT scan also showed well-marginated mass(n=6) with hemorrhagic necrosis(n--3) and contrast enhancement. One showed are shaped calcification along the capsule, and another case showed adhesion with aorta. In MRI, solid portion of the mass showed relatively low signal intensity on T1WI, increased signal on T2WI, and enhancement with gadolinium. Necrotic portion showed increased signal intensity on T1WI, and also showed increased signal intensity on T2WI, without contrast enhancement. Grossly the mass showed internal hemorrhagic necrosis of variable degree(n=6). CONCLUSION: If well-marginated mass is noted around the area of paraganglial distribution including organ of Zuckerkandl, especially with evidence of hemorrhagic necrosis and contrast enhancement, we must consider the possibility of paraganglioma despite no characteristic symptoms.
Aorta ; Gadolinium ; Humans ; Kidney ; Magnetic Resonance Imaging ; Necrosis ; Para-Aortic Bodies ; Paraganglioma* ; Tomography, X-Ray Computed

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.
Abdomen ; Epinephrine ; Fetus ; Ganglia ; Mediastinum* ; Mesenteric Artery, Inferior ; Neoplasm Metastasis ; Norepinephrine ; Para-Aortic Bodies ; Paraganglioma* ; Pheochromocytoma ; Transferases ; Urinary Bladder

Extraadrenal pheochromocytomas developed from the paraganglia other than adrenal medulla are uncommon tumors. Such lesions have been reported that as much as 10% of retroperitoneal paragliomas arise outside the adrenal gland. But their incidence and prognosis are changing partly as a result of progression in developmental mechanism, diagnostic tools and concepts including ultrastructure, immunohistochemistry. The ultrastructure of chromaffine neoplasm from adrenal and extraadrenal tissues has been studied with chrarnaffine granule specificity and type of catecholamine content. With rare exceptions, extraadrenal chrornaffine tumor mainly has high concentration of norepinephrine which is suggested as dict relationship to numoruos electrone dense granular bodies in the cell cytoplasrn. High or intermediate level of epinephrine in tissue is associated with cytoplasmic granular bodies of much less density, Recently we experienced the exception, a case of norepinephrine secreting pheochrom-ocytoma in the organ of Zuckerkandl with epinephrine granules in cytoplasm and this discrepancy probably due to the plasticity
Adrenal Glands ; Adrenal Medulla ; Cytoplasm ; Epinephrine ; Immunohistochemistry ; Incidence ; Norepinephrine ; Para-Aortic Bodies ; Pheochromocytoma ; Plastics ; Prognosis ; Sensitivity and Specificity

Retroperitoneal paraganglioma is a kind of uncommon tumor that occurs most commonly in the organ of Zuckerkandl located in the paraaortic area between the origin of the inferior mesenteric artery and the origin of the iliac artery. We experienced a case of retroperitoneal paraganglioma with an unusual location in a 52-year-old woman who had epigastric pain. Abdominal CT scan and ERCP showed a hypervascular mass near the distal CBD with bile duct dilatation. The surgically resected mass was diagnosed as paraganglioma. We report this unusual retroperitoneal paraganglioma, with a review of the literature.
Bile Ducts* ; Bile* ; Cholangiopancreatography, Endoscopic Retrograde ; Dilatation* ; Female ; Humans ; Iliac Artery ; Mesenteric Artery, Inferior ; Middle Aged ; Para-Aortic Bodies ; Paraganglioma* ; Tomography, X-Ray Computed

Pheochromocytoma is a hazardous and dramatic cause of hypertension. This potentially lethal neoplasm originates in most cases in the adrenal medulla and less frequently in the cells of the extraadrenal paraganglion system which are disseminated along the paravertebral axis from the pelvis to the base of the skull. The organ of Zuckerkandl is paraganglia lying the abdominal aorta with highest incidence in the region of the inferior mesenteric artery and usually degenerate shortly after birth. In the literature, and additional one case of pheochromocytoma arising from the organ of Zuckerkandl associated with intracerebral hemorrhage which was treated recently in the Kyung Hee University Hospital is presented in this report.
Adrenal Medulla ; Aorta, Abdominal ; Axis, Cervical Vertebra ; Cerebral Hemorrhage* ; Deception ; Hypertension ; Incidence ; Mesenteric Artery, Inferior ; Para-Aortic Bodies* ; Parturition ; Pelvis ; Pheochromocytoma* ; Skull