Extra-adrenal /retroperitoneal paraganglioma is a rare cause of hypertension in young with increased incidence of metastasis as compared to adrenal pheochromocytoma. We present a case of a young female with history of headache, nausea/vomiting, palpitations, uncontrolled hypertension, heat intolerance and diaphoresis. The 24-hour urine catecholamine levels were elevated. Clinical diagnosis of pheochromocytoma was made and further evaluation with Computed Tomography (CT) scan of the adrenals revealed extradrenal para-aortic retroperitoneal mass in keeping with paraganglioma. Gallium-68 DOTATE positron emission tomography-CT scan (PET-CT) confirmed the diagnosis without evidence of metastatic foci.
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