Baylor University Medical Center Proceedings July 2017 - 315

Figure 3. Surgical specimen of left adrenalectomy.

Figure 2. Computed tomography showing the adrenal mass.

adrenal mass (Figure 2). A 24-hour urinary vanillylmandelic acid
(VMA) was 311 mg/dL (normal range < 7 mg/dL) and metanephrines were 91,867 mcg/24 h (normal range 147-523 mcg/24 h).
The patient remained on veno-venous ECMO for 3 days.
During the hospitalization, blood pressures were labile and
temporary hemodialysis was required, but she then improved
without permanent end-organ damage. After 2 weeks of alpha blockade, the patient underwent an open adrenalectomy
(Figure 3). Blood pressure then became much easier to control.
Pheochromocytoma without local invasion was conﬁrmed histologically. The patient was discharged on carvedilol 25 mg
twice daily and at 6 months postoperatively was doing well with
a blood pressure of 108/71 mm Hg, normal renal function, and
normal 24-hour urinary metanephrines.
DISCUSSION
Pheochromocytoma is a catecholamine-secreting tumor arising from the chromaﬃn cells of the adrenal medulla. Present in
only 0.2% of all patients with hypertension in the United States
(1), these tumors typically occur sporadically, although they have
been associated with genetic disorders including multiple endocrine neoplasia syndrome type 2 (3). Due to the low prevalence
of pheochromocytoma and its often nonspeciﬁc signs and symptoms, diagnosis is often delayed. The classic triad of paroxysmal
hypertension, palpitations, and headaches is an unusual presentation of pheochromocytoma. Twenty-four-hour urinary VMA
and metanephrine testing remain the gold standard for diagnostic
testing. The sensitivity and speciﬁcity of 24-h urinary VMA and
metanephrines are 87.5% and 99.7%, respectively (4). About 10%
of pheochromocytomas are malignant, and surgical extirpation is
the only chance for a cure. However, the diagnosis of a malignant
pheochromocytoma may prove diﬃcult because they are histologiJuly 2017

cally identical to benign pheochromocytomas, and the only deﬁnitive proof of malignancy is local or distant invasion or metastasis.
Preoperative optimization is essential to the safe performance
of adrenalectomy (5). Alpha blockade with phenoxybenzamine
and prazosin and correction of intravascular volume contraction
are the essential tenets of preoperative management. Beta blockade for residual tachycardia may be added only after adequate
alpha blockade has been achieved to prevent unopposed alpha
adrenergic activity. Such unopposed alpha adrenergic activity
can lead to circulatory collapse.
The preoperative requirement of ECMO due to crisis, as in
our patient, is rare, but has been previously described (2, 6, 7).
The patient's clinical history and large adrenal mass pointed the
team toward the correct cause for her hypertensive emergency.
Early recognition led to early appropriate treatment and a successful outcome. This case highlights the diﬃculty in the management of cardiogenic shock and respiratory failure secondary
to pheochromocytoma and the important role that ECMO can
play in the successful resuscitation and management of these
patients and demonstrates a successful multidisciplinary approach in the management of a critically ill, complex patient.
1.

3.
4.

Stein PP, Black HR. A simpliﬁed diagnostic approach to pheochromocytoma. A review of the literature and report of one institution's experience.
Medicine 1991;70(1):46-66.
Chao A, Yeh YC, Yen TS, Chen YS. Phaeochromocytoma crisis-a
rare indication for extracorporeal membrane oxygenation. Anaesthesia
2008;63(1):86-88.
Pacak K, Eisenhofer G, Ilias I. Diagnosis of pheochromocytoma with special emphasis on MEN2 syndrome. Hormones (Athens) 2009;8(2):111-116.
Sheps SG, Jiang NS, Klee GG, van Heerden JA. Recent developments
in the diagnosis and treatment of pheochromocytoma. Mayo Clin Proc
1990;65(1):88-95.
Lenders JW, Duh QY, Eisenhofer G, Gimenez-Roqueplo AP, Grebe
SK, Murad MH, Naruse M, Pacak K, Young WF Jr, Endocrine Society.
Pheochromocytoma and paraganglioma: an Endocrine Society clinical
practice guideline. J Clin Endocrinol Metab 2014;99(6):1915-1942.
Suh IW, Lee CW, Kim YH, Hong MK, Lee JW, Kim JJ, Park SW, Park
SJ. Catastrophic catecholamine-induced cardiomyopathy mimicking acute
myocardial infarction, rescued by extracorporeal membrane oxygenation
(ECMO) in pheochromocytoma. J Korean Med Sci 2008;23(2):350-354.
Sojod G, Diana M, Wall J, D'Agostino J, Mutter D, Marescaux J. Successful
extracorporeal membrane oxygenation treatment for pheochromocytomainduced acute cardiac failure. Am J Emerg Med 2012;30(6):1017.e1-
1017.e3.

Hypertensive crisis secondary to pheochromocytoma

315

Table of Contents for the Digital Edition of Baylor University Medical Center Proceedings July 2017