Cardiac paraganglioma presenting as gestational hypertension

Abstract

A 24-YEAR-OLD WOMAN presented to an outside hospital during her third trimester of pregnancy with hypertensive episodes (230/130 mm Hg), headaches, and palpitations. Pre-eclampsia was excluded because of the absence of proteinuria. Additional evaluation revealed markedly elevated 24-h urinary catecholamines (norepinephrine, 978 mg/24 h [0–100]; normetanephrine, 4067 mg/24 h [50–650]; epinephrine, < 3 mg/24 h [0–25]; metanephrine, 108 mg/24 h [30–350]; and vanillylmandelic acid, 13.6 mg/d [0–7]), which confirmed the diagnosis of pheochromocytoma. A magnetic resonance image (MRI) of the abdomen showed a 2.8 3 1.9-cm mixed-intensity mass medial to the left adrenal gland, which was presumed to be a pheochromocytoma. The patient was brought to term safely on phenoxybenzamine therapy (10 mg twice daily), and a healthy baby was delivered via elective cesarean section. Postpartum, the patient underwent an elective left laparoscopic adrenalectomy. However, pathology revealed only normal adrenal tissue, and the patient’s clinical syndrome did not resolve. Genetic testing for a RET proto-oncogene mutation was negative, and the patient was referred for additional evaluation. During additional questioning, the patient reported having undergone prior surgery for a right neck mass, and her estranged father had been treated for a similar neck mass as well as an abdominal growth. Clinical suspicion for familial disease prompted functional imaging with F-DOPA