From the Guidelines
Yes, pheochromocytoma causes hypertension, as it is a rare form of secondary hypertension characterized by a highly heterogeneous clinical presentation, often discovered incidentally, and can lead to life-threatening complications if left untreated. Pheochromocytoma is a rare tumor that develops in the adrenal glands and produces excess catecholamines (primarily epinephrine and norepinephrine) 1. These hormones cause blood vessel constriction and increased heart rate, leading to hypertension. The hypertension from pheochromocytoma is often episodic or paroxysmal, with sudden, severe spikes in blood pressure accompanied by symptoms like headaches, sweating, and palpitations. However, about half of patients experience persistent hypertension.
The diagnosis typically involves measuring catecholamine levels in blood or urine, followed by imaging studies to locate the tumor. According to the 2024 ESC guidelines for the management of elevated blood pressure and hypertension, normetanephrine and metanephrine are preferred as screening tests for pheochromocytoma due to their constitutive secretion, as opposed to the highly variable nature of catecholamine secretion 1. Treatment usually requires surgical removal of the tumor, but patients need careful preoperative management with alpha-blockers (like phenoxybenzamine or doxazosin) for 1-2 weeks, followed by beta-blockers if needed, to control blood pressure and prevent hypertensive crisis during surgery 1.
Key points to consider in the management of pheochromocytoma include:
- Identifying a single tumor mandates surgical excision after adequate pharmacological preparation, as secreting pheochromocytomas can cause fatal events with no warning 1
- Administering doxazosin or terazosin, followed by a beta-blocker, usually controls BP and adrenergic crises 1
- Patients with pheochromocytoma have peripheral hypovolaemia that exposes them to the risk of profound hypotension, particularly right after tumor excision, and therefore require careful fluid administration management 1. Without treatment, pheochromocytoma can cause life-threatening complications including stroke, heart attack, or organ damage due to sustained or severe hypertension.
From the Research
Pheochromocytoma and Hypertension
- Pheochromocytoma is a catecholamine-secreting tumor that causes hypertension 2, 3, 4.
- The tumor secretes catecholamines such as norepinephrine, epinephrine, and dopamine, which can lead to severe hypertension and have devastating effects on multiple body systems 4.
- Hypertension in pheochromocytoma can be paroxysmal or sustained, and it is a key symptom that should raise suspicion for the disease 2, 3.
Characteristics of Hypertension in Pheochromocytoma
- High circulating levels of catecholamines can lead to severe hypertension, which can have devastating effects on multiple body systems 4.
- The signs and symptoms of pheochromocytoma are mostly due to hypercatecholaminemia, hypertension, complications, or coexisting diseases 2.
- Measurements of catecholamines and their metabolites in the plasma and urine may be normal between "attacks", and other conditions can elevate these values, making diagnosis challenging 2.
Treatment of Hypertension in Pheochromocytoma
- Surgical resection is the only modality of ultimate cure, but pharmacologic preoperative treatment remains the mainstay of successful outcome 4, 5.
- Alpha-adrenergic blocking agents, such as phenoxybenzamine, are used to control hypertension, and beta-adrenergic receptor blockade with propranolol may be indicated in patients with tachycardia and catecholamine-induced arrhythmias 6.
- Calcium channel blockers and/or beta-blockers may be used as alternatives or adjuncts to alpha-blockers in the preoperative treatment of pheochromocytoma 5.