Hypertensive Emergency Masquerading As Pheochromocytoma: A Report of Two Cases

Presentation Number: SUN 415
Date of Presentation: April 2nd, 2017

Raphael Hulkower*, Sriram Gubbi, Neva Castro, Wondim Teferi, Mimoza Meholli and Ulrich K Schubart
Jacobi Medical Center, Albert Einstein College of Medicine, Bronx, NY



While moderate elevations in metanephrines can be caused by medications, illness or stress, values in the range of 3-4 times the upper limit of normal are usually indicative of a pheochromocytoma/paraganglioma (PPGL) and warrant further imaging workup (1-2). We report two cases of hypertensive emergency with markedly elevated values in whom PPGL was subsequently ruled out.

Clinical Cases:

Case 1:

A 75 year old man with a history of HTN, CKD, and heart failure with preserved ejection fraction (EF) presented with a hypertensive emergency (BP 230/83, serum creatinine 2.9 mg/dL, previously 1.6, troponin-I 0.228 mcg/L without significant ECG changes). Testing on hospital day 2 revealed elevated plasma free normetanephrines (498 pg/ml, nl <148), and 24 hour urinary normetanephrines (2809 mcg/24 hrs, nl <560), and metanephrines (387 mcg, nl < 261). CT or MRI imaging were not obtained because of the patient’s renal dysfunction and the presence of a penile implant, respectively. An MIBG scan showed no evidence of PPGL. As an outpatient two weeks after initial testing, repeat plasma free metanephrines were within normal limits. Urinary normetanephrines remained minimally elevated.

Case 2:

A 56-year-old man with a history of untreated HTN presented with a one-week history of altered mental status, hallucinations, and shortness of breath and was found to have hypertensive emergency (BP 254/176 mmHg, serum creatinine 2.7 mg/dL, previously 1.0, Troponin-I 0.54 mcg/dL, ECG with no Q waves or ST changes, TTE with EF of 15% and diffuse hypokinesis). Testing on hospital day 3 showed plasma free normetanephrines (728 pg/ml, nl < 148), and metanephrines (76 pg/ml, nl <57), urinary normetanephrines (1533 mcg/g Cr, nl <282) and metanephrines (230 mcg/g Cr, nl < 158). Abdominal CT and octreotide scans revealed no findings consistent with PPGL. As an outpatient one month later the patient’s BP was well controlled and repeat plasma free normetanephrine and metanephrine levels were WNL.

Clinical Lessons:

To our knowledge, hypertensive emergency has not been reported as a cause of markedly elevated levels of plasma or urine metanephrines. Identifying hypertensive emergency as a cause of falsely elevated metanephrines – to levels seen in true PPGL – is important because patients presenting with severe hypertension may raise suspicion for PPGL. Our cases suggest that biochemical testing for PPGL in patients with hypertensive emergency should be delayed or repeated after resolution of the condition. This will help reduce patient harm from unnecessary imaging and provide high value care.


Nothing to Disclose: RH, SG, NC, WT, MM, UKS