Paraganglioma of the Organ of Zuckerkandl

Abstract

Paragangliomas are tumors of the sympathetic and parasympathetic paraganglia. While most paraganglioma are of parasympathetic origin and present as benign palpable masses of the neck, sympathetic paraganglioma are often secretory, presenting with symptoms related to excess catecholamines. Such symptoms include hypertension, headache, palpitations, and diaphoresis. Most sympathetic paraganglioma form within the adrenal medulla, the largest sympathetic paraganglia, and are commonly known as pheochromocytomas. However, sympathetic paragangliomas may present extra-adrenally, carrying a significantly higher risk of malignancy. In this manuscript, we examine a case of a young man with an extra-adrenal sympathetic paraganglioma of the Organ of Zuckerkandl. Furthermore, we discuss appropriate diagnostic workup and treatment of pheochromocytomas and sympathetic paragangliomas.