Pheochromocytoma of the organ of Zuckerkandl

Paragangliomas are uncommon neuroendocrine neoplasms that occur in characteristic locations. While parasympathetic paragangliomas are mainly located at the head and neck, sympathetic paragangliomas are mostly located below the neck. Among parasympathetic paragangliomas, pheochromocytomas are the most common. Ninety percent of cases of pheochromocytomas arise within the adrenal gland. We report a case of a 63-year-old woman with an extra-adrenal pheochromocytoma of the organ of Zuckerkandl detected by CT and MRI and subsequently confirmed by postoperative histology and immunohistochemistry.