From the Research
Pheochromocytoma can cause chronic piloerection in some patients due to the excessive secretion of catecholamines, which activate alpha-adrenergic receptors in the arrector pili muscles attached to hair follicles, leading to piloerection. This occurs as part of the sympathetic nervous system activation that characterizes pheochromocytoma, a tumor of the adrenal gland that secretes excessive amounts of catecholamines, primarily epinephrine and norepinephrine 1.
Key Points
- Pheochromocytomas are rare, mostly benign catecholamine-producing tumors of chromaffin cells of the adrenal medulla or of a paraganglion 2.
- Typical clinical manifestations include sustained or paroxysmal hypertension, severe headaches, palpitations, and sweating resulting from hormone excess 2.
- Chronic piloerection is less common than these classic symptoms but can be a persistent manifestation in some cases.
- Definitive treatment involves surgical removal of the tumor after appropriate alpha and beta-adrenergic blockade to control blood pressure and prevent complications 3, 2.
Diagnosis and Treatment
The diagnosis of pheochromocytomas depends mainly upon the demonstration of catecholamine excess by 24-h urinary catecholamines and metanephrines or plasma metanephrines 2. They are localized by a computed tomography scan and magnetic resonance imaging of the adrenal glands and abdomen; complementary 123I-metaiodobenzylguanidine scintigraphy and 18F-dihydroxyphenylalanine-positron emission tomography are available 2. Laparoscopic and adrenal sparing surgical intervention following preoperative alpha-blockade is the treatment of choice and usually curative 2.
Importance of Early Detection
If not properly diagnosed, secretion of catecholamines may lead to fatal cardiovascular consequences 4. Biochemical testing for pheochromocytoma should be performed not only in symptomatic subjects or in subjects with adrenal incidentaloma but also in subjects with a genetic predisposition for pheochromocytoma 4. After tumor removal, further follow-up is necessary due to possible recurrence 4. Although prognosis after tumor resection is excellent, a significant proportion of pheochromocytomas recur, some as metastases 4. Thus, appropriate follow-up is mandatory.