From the Guidelines
Pheochromocytoma is a rare tumor that develops in the adrenal glands, causing excessive production of hormones like adrenaline and noradrenaline, leading to episodes of high blood pressure, rapid heartbeat, and other symptoms. This condition is characterized by increased blood pressure variability, which constitutes an additional independent risk factor for cardiovascular morbidity and mortality, as noted in studies 1. The diagnosis of pheochromocytoma should be entertained in a hypertensive patient with a combination of headaches, palpitations, and sweating, typically occurring in an episodic fashion, with a diagnostic specificity of 90% 1.
Key Characteristics of Pheochromocytoma
- Rare tumor of the adrenal glands
- Excessive production of catecholamines (adrenaline and noradrenaline)
- Episodes of high blood pressure, rapid heartbeat, severe headaches, sweating, and anxiety
- Usually benign (non-cancerous) but can be dangerous if left untreated
Importance of Prompt Diagnosis and Treatment
The main treatment for pheochromocytoma is surgery to remove the tumor, but before surgery, patients typically need medication to control blood pressure and heart rate, as outlined in guidelines 1. Controlling the excessive hormone production can prevent serious and potentially life-threatening complications, such as heart attacks, strokes, or other serious complications. Common medications include alpha-blockers like phenoxybenzamine or doxazosin, which are started first to control blood pressure, followed by beta-blockers like propranolol if needed for heart rate control.
Recent Guidelines and Recommendations
According to the most recent guidelines 1, phaeochromocytomas are rare adrenal tumours that secrete catecholamines and are present in <0.2% of patients with hypertension. The guidelines recommend identifying a single tumour and mandating surgical excision after adequate pharmacological preparation, because secreting PPGLs can cause fatal events with no warning. Administering doxazosin or terazosin, followed by a beta-blocker, usually controls BP and adrenergic crises.
From the Research
What is Pheochromocytoma
- Pheochromocytoma is a rare type of tumor that produces catecholamines, such as adrenaline, noradrenaline, and dopamine 2, 3.
- These tumors originate from the chromaffin cells of the adrenal medulla or from extra-adrenal chromaffin tissue, and are also known as paragangliomas (PGL) 2.
- The secretion of catecholamines by these tumors can lead to life-threatening hypertensive crises 2.
Characteristics of Pheochromocytoma
- Approximately 30-40% of pheochromocytomas have a hereditary background due to mutations of known susceptibility genes 2, 3.
- The tumors can be diagnosed through biochemical testing, measuring catecholamines or their metabolites in plasma or urine 3.
- Molecular testing, functional imaging, and targeted therapies have improved diagnostic precision and management of pheochromocytomas 3.
Treatment and Management
- Surgery with minimal invasive procedures is a recommended therapeutic approach after pretreatment with an alpha receptor blocking medication 2.
- Recent advances in genetic research have led to the development of personalized treatment approaches based on genetic profiling 3.
- Targeted therapies, such as radionuclide therapy with lutetium-177 DOTATATE, and systemic therapies like sunitinib and cabozantinib, offer potential new options for patients with pheochromocytoma 3.