Introduction

A pheochromocytoma is a rare, catecholamine-secreting tumor derived from chromaffin cells.

When such tumors arise outside of the adrenal gland, they are termed extra-adrenal
pheochromocytomas, or paragangliomas. (See the images below.) Because of excessive
catecholamine secretion, pheochromocytomas may precipitate life-threatening hypertension or
cardiac arrhythmias. If the diagnosis of a pheochromocytoma is overlooked, the consequences
can be disastrous, even fatal; however, if a pheochromocytoma is found, it is potentially
curable.
[1]

Axial, T2-weighted magnetic resonance imaging (MRI) scan showing large left suprarenal mass of high signal
intensity on a T2-weighted image. The mass is a pheochromocytoma.

Abdominal computed tomography (CT) scan demonstrating left suprarenal mass of soft-tissue attenuation
representing a paraganglioma
The term pheochromocytoma (in Greek, phios means dusky, chroma means color, and cytoma
means tumor) refers to the color the tumor cells acquire when stained with chromium salts. Roux
performed the first surgical resection of a pheochromocytoma in Lausanne, Switzerland in 1926.
Later the same year, Charles Mayo performed the first surgical resection in the United States.
[2]
In a study by Paraby et al, severe hypertension was associated with 5 of 35 pheochromocytoma
resections (14.3%), compared with 2 of 106 nonpheochromocytoma adrenal tumor resections
(1.9%), and there was a significant increase in the need for intraoperative hypertensive treatment
in the pheochromocytoma patients. In the study, the researchers investigated whether significant
differences exist between perioperative hemodynamic changes arising from laparoscopic
adrenalectomy for pheochromocytomas and those stemming from the use of the procedure for
other types of adrenal tumors. The study included 34 patients who underwent laparoscopic
adrenalectomy for pheochromocytomas (total resections = 35) and 104 patients who underwent
the laparoscopic adrenalectomy for other tumors (total resections = 106).
[3]
Despite the greater occurrence of hypertension in the pheochromocytoma group, the
investigators found no instances of transient or persistent systolic blood pressure of greater than
220 mm Hg in either group and noted no significant differences in recovery room hemodynamic
parameters, frequency of persistent hypotension, or occurrence of heart rates greater than
120/min between the 2 groups.
Over 90% of pheochromocytomas are located within the adrenal glands, and 98% are within the
abdomen. Extra-adrenal pheochromocytomas develop in the paraganglion chromaffin tissue of
the sympathetic nervous system. They may occur anywhere from the base of the brain to the
urinary bladder. Common locations for extra-adrenal pheochromocytomas include the organ of
Zuckerkandl (close to origin of the inferior mesenteric artery), bladder wall, heart, mediastinum,
and carotid and glomus jugulare bodies
3
.

1. Waguespack SG, Rich T, Grubbs E, et al. A Current Review of the Etiology, Diagnosis, and
Treatment of Pediatric Pheochromocytoma and Paraganglioma. J Clin Endocrinol Metab. Mar 9
2010;[Medline].
2. Sheps SG, Jiang NS, Klee GG, van Heerden JA. Recent developments in the diagnosis and
treatment of pheochromocytoma. Mayo Clin Proc. Jan 2010;65(1):88-95. [Medline].
3. Parnaby CN, Serpell MG, Connell JM, et al. Perioperative haemodynamic changes in patients
undergoing laparoscopic adrenalectomy for phaeochromocytomas and other adrenal tumours.
Surgeon. Feb 2010;8(1):9-14. [Medline].