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Posterior mediastinal Paraganglioma (Extraadrenal Pheochromocytoma)
Characteristics:
inhomogeneous mass with small areas of calcification in left paraspinal area
Extraadrenal pheochromocytoma is termed paraganglioma that may be benign or malignant
Thoracic paragangliomas are rare: they account only 1-2% of all cases of pheochromocytoma
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1) in the area of aortic arch or main pulmonary artery;
2) in the sympathetic chain in posterior mediastinum; and
3) in or adjacent to the heart.
They are seen as rounded soft tissue masses that are usually hypervascular and therefore enhance brightly at CT after IV administration of contrast media.
Arteriography reveals enlarged feeding vessels, pathologic vessels with the tumor, and an intense tumor blush.
Radioiodine metaiodobenzylguanidine (MIBG) shows increased activity in paragagliomas and is an useful method of identifying extraadrenal pheochromocytoma.
MIBG is a noradrenalin analogue that is stored by most functional paragangliomas within intracellular storage granules of the presynaptic adrenergic neurons.
For adrenal pheochromocytomas, 131I-MIBG scintigraphy has
a sensitivity ranging from 77% to 90% and a speciicity of 95% to 100%.
Carney's triad consists of extraadrenal paragaglioma, gastric leiomyosarcoma, and pulmonary chondroma
In some cases, complete surgical excision and cure are possible, but in others local invasion or extensive bleeding during surgery precludes complete excision.
In the latter patients, the neoplasm usually recurs although growth rate is often slow.
Metastases have been documented in about 10% of patients.
첨부파일은 case report 된 case 입니다.
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