What is pheochromocytoma (adrenal gland tumor)?

From the Guidelines

Pheochromocytoma should be treated with surgical removal of the tumor after careful preparation with alpha-blockers, such as doxazosin or terazosin, followed by beta-blockers if needed, to control blood pressure and adrenergic crises, as recommended by the most recent guidelines 1. The diagnosis of pheochromocytoma typically involves blood and urine tests to measure catecholamine levels, followed by imaging studies like CT scans or MRIs to locate the tumor. Key points to consider in the management of pheochromocytoma include:

• The importance of alpha-blocker therapy for 1 to 3 weeks prior to surgery to prevent a catecholamine surge intraoperatively 1
• The need for adequate fluid administration to prevent post-operative hypotension, as patients with pheochromocytoma often have peripheral hypovolaemia 1
• The recommendation for genetic testing to identify potential hereditary causes, as pheochromocytoma can be part of genetic syndromes like MEN2 or von Hippel-Lindau disease 1
• The use of plasma free metanephrines as the best screening test for pheochromocytoma, with a 99% sensitivity and an 89% specificity 1 It is crucial to prioritize the most recent and highest quality study, which in this case is the 2024 ESC guidelines for the management of elevated blood pressure and hypertension 1, to ensure the best possible outcome for patients with pheochromocytoma in terms of morbidity, mortality, and quality of life.

From the Research

Definition and Overview of Pheochromocytoma

• Pheochromocytoma is a rare, mostly benign catecholamine-producing tumor of chromaffin cells of the adrenal medulla or of a paraganglion 2.
• It is an uncommon cause of secondary hypertension, but it is also a curable form of hypertension with proper treatment 3.
• The outlook for patients with pheochromocytoma is excellent if diagnosed and treated properly, but it can cause serious complications and death if undiagnosed or untreated 3.

Clinical Manifestations

• Typical clinical manifestations of pheochromocytoma include sustained or paroxysmal hypertension, severe headaches, palpitations, and sweating resulting from hormone excess 2.
• The presentation of pheochromocytoma is highly variable and can mimic many other diseases, making it challenging to diagnose 3, 2.
• The spectrum of clinical manifestations is wide, and pheochromocytoma may mimic a variety of common disorders 3.

Diagnosis

• The diagnosis of pheochromocytoma depends mainly on the demonstration of catecholamine excess by 24-h urinary catecholamines and metanephrines or plasma metanephrines 2.
• Localization of the tumor is done by computed tomography scan and magnetic resonance imaging of the adrenal glands and abdomen, with complementary 123I-metaiodobenzylguanidine scintigraphy and 18F-dihydroxyphenylalanine-positron emission tomography available 2.
• A high index of clinical suspicion coupled with careful clinical evaluation and laboratory testing is key to reaching the diagnosis 3.

Treatment

• Surgical removal of pheochromocytoma is the treatment of choice, with laparoscopic and adrenal sparing surgical intervention following preoperative alpha-blockade being the preferred method 2.
• For patients with inoperable, malignant, recurrent, or multicentric pheochromocytomas, chronic medical therapy is indicated in the form of alpha- and/or beta-blockade or inhibition of catecholamine synthesis with alpha-methyl-para-tyrosine 3, 4.
• Pharmacologic management, including alpha-adrenergic blockers, beta-adrenergic blockers, and alpha-methylparatyrosine, plays a crucial role in the management of pheochromocytoma 4, 5.