Paragangliomas (extra-adrenal pheochromocytomas) are rare
tumors originating in the autonomic nervous system, whose clinical manifestations are the result of excessive production of
catecholamines. The classic triad of palpitations,
headache, and diaphoresis associated with
hypertension (HT), elevated serum and urine
catecholamine levels, and visualization of the mass on imaging tests, enables diagnosis of these
tumors, which are mostly benign, and if diagnosed and treated early, are a potentially treatable cause of secondary
hypertension. We present the case of a 28-year-old woman, a former smoker, sedentary, with a family history of HT, diabetes and
coronary heart disease, and a personal history of episodic
headaches, diaphoresis and
syncope. HT was diagnosed at the age of 18 and controlled with
losartan 50 mg/day up to the age of 28, when she was referred to a specialist for "worsening HT"/suspected secondary HT. The patient had no major alterations on physical examination except casual BP of 140/95 mmHg. On further study, ABPM recording revealed a non-dipper profile, with higher than normal blood pressure values and two blood pressure peaks without associated symptoms. She had elevated plasma
catecholamine levels and a hypervascular retroperitoneal mass was visualized on CT and MRI, anterior to the inferior vena cava, measuring about 8 x 5.5 x 4.5 cm, with calcifications and areas of
necrosis, confirmed by 123(I-MIBG) scintigraphy and PET scan. The mass was excised by
laparotomy and the diagnosis of
paraganglioma was confirmed. In postoperative follow-up the patient's blood pressure and
catecholamine levels had normalized.