SAT-189 Pheochromocytoma as a Rare Cause of Elevated Blood Pressure During Pregnancy

Endocrine causes of elevated blood pressure during pregnancy are considered a rare, mainly when compared to the more frequent pregnancy-related hypertensive disorders. Thus, the diagnosis of endocrine-related secondary hypertension cases and the outcome of pregnancy will largely depend upon a high clinical suspicion. A 28 year-old female, presented in pregnancy with elevated, difficult to control blood pressure, leading to induced delivery of a preterm 32 week baby. The baby was admitted to the NICU, where she stayed for 3 weeks. After delivery, Patient was evaluated in the Endocrine Clinic. She reported episodes of pulsatile headaches, palpitations and excessive sweating for the past 1 year. Each episode would last around 10 minutes and resolved spontaneously. Systolic blood pressure during these episodes was in the 200s and would return back to normal afterwards. Past medical, surgical and social history was unremarkable. She reported that her mother died suddenly in her 30s from an unknown cause. Physical exam, including blood pressure, was unremarkable in the clinic. The patient underwent a work up for secondary causes of hypertension after delivery. Tests included plasma metanephrines: 327pg/ml (<57pg/ml), plasma normetanephrines: 7,135pg/ml (<148pg/ml), Chromogranin A 768 ng/ml (<93ng/ml) and a 24 Hour fractionated total metanephrines: 10,170 mcg/24hrs (40-412mcg/24h). She had normal TSH, aldosterone, renin and AM cortisol level. CT scan of chest, abdomen and pelvis showed a large right retroperitoneal, heterogeneously enhancing mass, measuring 5.8 x 3.7 x 8.3 cm, which encircles the aorta approximately 180 degrees, without evidence of vascular invasion. Diltiazem three times a day was initiated for preoperative blood pressure control. After 2 weeks, she underwent laparoscopic resection. Pathology showed lesional cells that are positive for synaptophysin and chromogranin while negative for AE1/3 and sustentacular cells that are highlighted by S100, thus supporting a diagnosis of paragangliomas. Postoperatively, the patient was weaned off all blood pressure medications. Repeat testing showed plasma metanephrines: 26 pg/ml and plasma normetanephrines: 164 pg/ml. The patient is scheduled to establish care with Endocrine Genetics Clinic. A timely diagnosis of pheochromocytoma during pregnancy can dramatically affect maternal and fetal mortality rates (1). This case illustrates the importance of a high clinical suspicion for pheochromocytoma as a cause of elevated blood pressure during pregnancy, particularly in patients who lack other characteristics typical of pregnancy related hypertension disorders (proteinuria, edema, etc.) or present with an elevated blood pressure early in pregnancy. References: (1) Van der Weerd et al., Pheochromocytoma in pregnancy: case series and review of literature. European Journal of Endocrinology (2017) 177, R49–R58