“…Pheochromocytoma and secreting extra-adrenal paraganglioma are the cause of the secondary hypertension in approximately 0.1% of hypertensive patients and the prevalence is estimated to be 1 per 100,000 persons per year or less [ 7 ]. Unlike pheochromocytoma, which have been described as having a 10% malignancy rate, paraganglioma, especially the abdominal extra-adrenal have a higher rate of malignancy ranging from 14% to as high as 50% [ 1 ] and retroperitoneal paragangliomas have a more genetic background [ 1 , 3 , 4 , 8 ]. Therefore genetic testing should be offered to patients diagnosed with extra-adrenal paraganglioma, particularly in patients who are young, have multiple tumors, or have a family history of malignancy [ 3 ].…”