From the Guidelines
Paraganglioma is another name for pheochromocytoma, though there is a subtle distinction between the two terms, with pheochromocytoma referring to tumors in the adrenal medulla and paraganglioma referring to extra-adrenal tumors. The most recent and highest quality study, 1, published in 2024, provides guidance on the management of elevated blood pressure and hypertension, including the diagnosis and treatment of pheochromocytoma and paraganglioma. According to this study, pheochromocytomas are rare adrenal tumors that secrete catecholamines and are present in less than 0.2% of patients with hypertension.
Key Points
- Pheochromocytoma specifically refers to a rare neuroendocrine tumor that develops in the adrenal medulla, while paraganglioma refers to similar tumors that arise from extra-adrenal chromaffin cells located along the sympathetic and parasympathetic chains.
- Both tumors secrete excessive amounts of catecholamines, which can cause episodic or persistent hypertension, headaches, sweating, palpitations, and anxiety.
- The distinction between these terms is anatomical, with pheochromocytomas located within the adrenal glands and paragangliomas developing outside the adrenal glands.
- Despite this technical difference, in clinical practice, the term pheochromocytoma is sometimes used broadly to refer to both adrenal and extra-adrenal tumors, though specialists typically prefer the more precise terminology, as noted in 1, 1, and 1.
Diagnosis and Treatment
The study 1 recommends screening for mutations that can drive management of the proband and the family, as well as inform the choice of functional imaging. It also suggests that identifying a single tumor mandates surgical excision after adequate pharmacological preparation, and that administering doxazosin or terazosin, followed by a beta-blocker, usually controls blood pressure and adrenergic crises.
Management
Adequate fluid administration should be carefully managed to avoid profound hypotension, particularly right after tumor excision, as patients with pheochromocytoma or paraganglioma have peripheral hypovolaemia that exposes them to this risk, as noted in 1.
From the Research
Alternative Names for Pheochromocytoma
- Paraganglioma (in cases where the tumor originates from extra-adrenal chromaffin tissue) 2
- No other specific alternative names are mentioned in the provided studies, but pheochromocytomas are also referred to as catecholamine-secreting tumors or neuroendocrine tumors 3, 4, 5, 6
Key Characteristics
- Pheochromocytomas are rare neuroendocrine tumors that arise from chromaffin cells in the adrenal medulla or extra-adrenal sites 3, 2, 4, 5, 6
- They are characterized by the production of catecholamines, including epinephrine, norepinephrine, and dopamine 3, 2, 4, 5
- Approximately 30-40% of pheochromocytomas have a hereditary background due to mutations of known susceptibility genes 2, 5, 6