Postoperative Management in Patients with Pheochromocytoma and Paraganglioma

Abstract

Pheochromocytomas and paragangliomas (PPGLs) are rare catecholamine-secreting neuroendocrine tumors of the adrenal medulla and sympathetic/parasympathetic ganglion cells, respectively. Excessive release of catecholamines leads to episodic symptoms and signs of PPGL, which include hypertension, headache, palpitations, and diaphoresis. Intraoperatively, large amounts of catecholamines are released into the bloodstream through handling and manipulation of the tumor(s). In contrast, there could also be an abrupt decline in catecholamine levels after tumor resection. Because of such binary manifestations of PPGL, patients may develop perplexing and substantially devastating cardiovascular complications during the perioperative period. These complications include hypertension, hypotension, arrhythmias, myocardial infarction, heart failure, and cerebrovascular accident. Other complications seen in the postoperative period include fever, hypoglycemia, cortisol deficiency, urinary retention, etc. In the interest of safe patient care, such emergencies require precise diagnosis and treatment. Surgeons, anesthesiologists, and intensivists must be aware of the clinical manifestations and complications associated with a sudden increase or decrease in catecholamine levels and should work closely together to be able to provide appropriate management to minimize morbidity and mortality associated with PPGLs.

Keywords: arrhythmia; hypertension; hypotension; pheochromocytoma; postoperative.

Figure 1

Postoperative management of hypertension following tumor resection. ^# Residual or metastatic disease causing an increased blood pressure is treated using α–adrenoceptor blocker. If necessary, β-adrenoceptor blocker and/or calcium channel blocker is added. β-adrenoceptor blocker might be used at first for epinephrine-secreting tumors. ^∞ Management of hypertensive emergency. * Underlying essential hypertension is treated according to currently accepted guidelines. ^$ Phentolamine is used to manage hypertensive crisis or in cases of resistant hypertension. BP, blood pressure; IV, intravenous; PO, per oral.

Figure 2

Postoperative management of hypotension following tumor resection. * In the differential diagnosis of hypotension consider downregulation of adrenoceptors, cardiogenic shock, sepsis, and medication-induced. BP, blood pressure.

Figure 3

Management of tachyarrhythmia following PPGL resection in the postoperative period. ^# Unstable tachyarrhythmia implies patient has tachyarrhythmia along with hemodynamic instability or concerning symptoms. ^Ŧ Treatment in intensive care unit usually begins with calcium channel blockers. If heart rate is not well controlled, β-adrenoceptor blockers such as esmolol or metoprolol is added. In patients with increased blood pressure along with increased heart rate, use of combined α– and β-adrenoceptor blocker such as labetalol is recommended. Moreover, β-adrenoceptor blocker might be used at first for epinephrine-secreting tumors. * Underlying causes for increased catecholamines include their release during manipulation / resection of tumor and residual/ metastatic disease (patient must be on appropriate adrenoceptor blockade). ^$ Other causes include inotropes used to correct postoperative hypotension, rebound tachycardia by discontinuation of β-adrenoceptor blockers used preoperatively as well as anemia, hypovolemia, pain, and anxiety. ^@α-adrenoceptor blocker are used in the presence of residual or metastatic disease. BPM, beats per minute; EKG, electrocardiogram; IV, intravenous; PO, per oral.

Figure 4

Management algorithm of postoperative NSTEMI. ^# Aspirin and clopidogrel is given in the postoperative period only when it is safe from surgical point of view. CTA, computed tomography angiography; 2D-ECHO, two-dimensional echocardiography; EKG, electrocardiography; NSTEMI, non-ST-elevation myocardial infarction; NTG, nitroglycerin.