Multiple Hereditary Paraganglioma and Pheochromocytoma Syndrome

32-year-old female marathon runner who presented with uncontrolled hypertension and recurrent episodes of vertigo. A series of contrast enhanced MRA images of the neck are provided demonstrating a circumscribed, solid appearing, avidly enhancing masses. There are lesions involving the prevascular space between the left common carotid and subclavian arteries, more superiorly at the level of the left carotid bifurcation, with additional subcentimeter lesions along the left and right internal carotid arteries. The largest lesions demonstrate prominent internal vessels. On the time of flight MRA of the neck, prominent flow voids were identified within the lesions. The 3-D maximal intensity projection MRA image demonstrates the left carotid bifurcation lesion nicely. The findings are compatible with multiple paragangliomas. The patient was given a diagnosis of multiple hereditary paraganglioma and pheochromocytoma syndrome. Paraganglioma may also be seen in the setting of VHL, MEN-2, NF-1, mitochondrial succinate dehydrogenase mutations, tuberous sclerosis, Carney triad and Sturge-Weber.