Laparoscopic unilateral adrenalectomy is the procedure of choice for most pheochromocytomas [15]. Hypertensive crisis from catecholamine release during tumor manipulation is the feared complication [10]. Intravenous infusion of nicardipine or nitroprusside, a vasodilating agent, is the preferred intraoperative BP agents. In patients who may not have adequate pre-operative α-adrenergic blockage, the intravenous α-adrenergic antagonist phentolamine may be used (Table 23.1) [24].

Anesthetic agents known to stimulate catecholamine secretion such as fentanyl, morphine, ketamine, halothane, and desflurane must be avoided, whereas propofol, barbiturates, synthetic opioids, etomidate, and the other anesthetic gases are safe [23]. Intravenous volume expansion is often begun preoperatively provided cardiac and renal function will tolerate. Intravenous insulin may also be required for catecholamine-induced hyperglycemia.

Immediately postoperative, approximately 50 % of patients have sustained hypertension [28], whereas others experience hypotension, usually a multifactorial response to sudden catecholamine depletion, hypovolemia, and persistence of α-adrenergic blockade. Intravascular volume replacement followed by pressor support is the cornerstone of management.

Complete tumor resection should be assessed 2–6 weeks postoperatively with collection of plasma or urine metanephrines [15, 23]. Normal values do not exclude the possibility of remaining microscopic disease; therefore, biochemical screening must be performed annually, although no further imaging is required. Residual essential hypertension should be managed following the seventh report of the Joint National Committee (JNC 7) hypertension guidelines [29].

Persistent metanephrine elevations may signal residual tumor, multifocal disease, or metastatic disease and must be followed by CT, MRI, or functional imaging. Hypertension and symptoms of catecholamine excess should be managed as they were preoperatively, primarily with α-adrenergic blockade; however, the selective α-blocking agents are typically employed in these cases to minimize the side effects of expected long-term use.

In patients with refractory disease, such as those with residual or metastatic disease, metyrosine, a tyrosine hydroxylase inhibitor, can be used [23]. The side effects of metyrosine can be disabling, including extrapyramidal symptoms, depression, galactorrhea, and sedation, particularly with long-term use. Therefore, its use should be reserved for widespread disease with intractable symptoms despite α-adrenergic blockade (Table 23.1).

Finally, genetic testing should be discussed at the initial postoperative visit in any patient with a family history of pheochromocytoma, a paraganglioma, younger than 45 years, a malignant tumor, bilateral or multicentric disease, or clinical presentation consistent with one of the known hereditary syndromes [30, 31]. Our patient’s presentation with pheochromocytoma, medullary thyroid cancer, and primary hyperparathyroidism was consistent with MEN 2a, which was ultimately confirmed with gene testing. His diseased father, who had a pheochromocytoma at autopsy, was likely affected as well. Prior knowledge of his father’s medical history may have prompted increased screening in our patient with early detection of pheochromocytoma. The health of the patient’s affected daughter, however, has benefited. She underwent prophylactic thyroidectomy within weeks of diagnosis at age 2 and the pathology revealed C-cell hyperplasia. She will undergo annual screening for pheochromocytoma in hopes of preventing the complications suffered by her father and grandfather.

Pheochromocytomas are rare, potentially fatal tumors that may be clinically silent or present with hypertensive emergencies, sustained arrhythmias, or sudden death. The manifestations of pheochromocytoma result from catecholamine excess, which is variable based on the predominant catecholamine released and the adrenergic receptor affected. Our patient experienced episodic hypertension, which was consistent with the markedly elevated epinephrine secretion of his tumor, and ultimately presented with a hypertensive emergency. His tumor was identified immediately on presentation, and blood pressure control was achieved with α-blockade, followed by heart rate control with β-blockade. Fortunately, his previous presentation, during which he underwent cardiac catheterization, did not precipitate a crisis. This highlights the need for a high index of suspicion for this sometimes elusive tumor, particularly in patients who experience paroxysms of symptoms, in young patients with hypertension, or in patients with a suspicious family history. The coexisting medullary thyroid cancer and primary hyperparathyroidism were consistent with MEN 2a, and recognition of this enabled gene testing of the patient’s family, ultimately resulting in the identification and prophylactic treatment of his affected daughter.