Pseudopheochromocytoma Definition, Symptoms, Causes, Treatment | Pseudopheochromocytoma vs Pheochromocytoma

What is Pseudopheochromocytoma?

Pseudopheochromocytoma is distinguished by severe symptomatic paroxysmal hypertension (HT), which is identical to the clinical presentation of pheochromocytoma but without an adrenal tumor on imaging studies and with normal catecholamine values. 

Pseudopheochromocytoma is a rare condition with an uncertain etiology, antihypertensive treatment is typically ineffective, and a large proportion of patients develop chronic disability. Invasive percutaneous radiofrequency ablation of the sympathetic nervous system through a catheter placed at the level of the renal arteries is recently established as a treatment for HT that is resistant to standard medical treatment.

Pseudopheochromocytoma Definition

Pseudopheochromocytoma, also called paroxysmal sympathetic hyperactivity or sympathetic storm, is a disease in which the sympathetic nervous system is overactive at times. It resembles pheochromocytoma, an adrenal gland tumor that secretes excessive levels of the hormones noradrenaline and adrenaline, and exhibits some of the same clinical characteristics. The symptoms of pseudo pheochromocytoma, on the other hand, are caused by a variety of underlying illnesses or triggers rather than an adrenal tumor.

Pseudopheochromocytoma Symptoms

Pseudopheochromocytoma is a syndrome characterized by tremors, anxiety, sweating, and palpitations as well as paroxysmal, severe hypertension. In certain cases, additional symptoms, such as chest discomfort, headache, nausea, dizziness, and even pseudoseizures and strangely low blood pressure, are also present. 

Pseudo-pheochromocytoma exhibits several same clinical manifestations as pheochromocytoma; however, the key difference is that no structural or biochemical abnormality is present; yet, there is evidence of mild to moderate catecholamine excess, especially during paroxysms.

Pseudopheochromocytoma Causes

The physiopathology of pseudo pheochromocytoma isn't known yet, but the main cause is thought to be the activation of the sympathetic nervous system, which causes more dopamine and epinephrine to be released and makes the adrenergic receptors more sensitive. This is often caused by an emotional factor. 

Patients with this illness were initially classified as having altered autonomic nervous system function or anomalies in catecholamine distribution and the subsequent impact on target organs. Page first proposed this idea in 1935, when he reported a group of ladies suffering from paroxysmal hypertension and experiencing flushing, sweating, and palpitations. These symptoms are thought to be secondary to panic attacks, causing "irritation" of sympathetic and parasympathetic centers in the diencephalon. Later in the 1980s, Kuchel discovered that high DA levels are a sign of heightened SNS activation in Pseudopheochromocytoma patients. 

Pseudopheochromocytoma Treatment

Treatment is usually complicated and uses three methods: Psychopharmacologic treatment (antidepressants and benzodiazepines), antihypertensive treatment (usually a regimen of alpha- and beta-blockers is suggested), and psychotherapy, which doesn't work for at least 40% of patients. Sympathetic nervous system ablation is possibly a treatment option in a subgroup of patients with refractory HT and paroxysmal crises coupled with proven sympathetic hyperactivity.

Pseudopheochromocytoma vs Pheochromocytoma

Pheochromocytoma and pseudo-pheochromocytoma share paroxysmal hypertension as a presenting symptom, but their underlying causes are different. Pheochromocytoma is a rare tumor of the adrenal gland that produces an excess of catecholamines, whereas pseudo-pheochromocytoma is not associated with catecholamine excess. Pseudopheochromocytoma is hypothesized to be influenced by emotional factors and episodic dopamine release.