Pheochromocytoma: Symptoms, Diagnosis, Treatment & Surgery

Introduction

Pheochromocytoma is an uncommon but serious tumor that develops in the adrenal glands. Excessive catecholamines produced by these tumors cause high blood pressure and other problems. Pheochromocytomas are usually benign but can create serious health conditions if not treated. This article discusses the symptoms, diagnosis, and treatment methods, including surgery.

What Is Pheochromocytoma?

Pheochromocytoma is a neuroendocrine tumor that arises in the adrenal medulla, the inner region of the adrenal glands. These tumors produce excess amounts of adrenaline (epinephrine) and noradrenaline (norepinephrine), hormones that are involved in the control of heart rate, blood pressure, and responses to stress. Although the majority of pheochromocytomas are benign, a few can be malignant and spread to other tissues in the body.

Symptoms of Pheochromocytoma

The presentation of pheochromocytoma is largely because of excessive hormone secretion. The manifestations tend to happen in waves and can include:

• Elevated blood pressure (hypertension) – Episodic or fixed
• Severe headache – Characteristically pulsating and usually accompanied by hypertension
• Excessive sweating (diaphoresis) – Without exertion
• Rapid heart rate (tachycardia) – Palpitations or arrhythmias
• Anxiety or panic – Resulting from excessive release of adrenaline
• Tremors and shaking – Due to nervous system overstimulation
• Unexplained weight loss – In the presence of normal or enhanced appetite
• Fluctuating blood pressure levels – May cause dizziness and fainting

Symptoms may be provoked by stress, physical activity, surgery, or some medications. If not treated, pheochromocytoma can result in life-threatening conditions like heart attack or stroke.

Diagnosis of Pheochromocytoma

Pheochromocytoma is diagnosed through a series of tests to identify excessive catecholamines and determine the site of the tumor.

1. Biochemical Testing

• Plasma Free Metanephrines Test: Assesses hormone metabolites in the blood.
• 24-Hour Urine Metanephrine Test: Tests for catecholamine metabolites throughout a whole day.

2. Imaging Studies

• CT Scan (Computed Tomography): Assists in finding adrenal tumors.
• MRI (Magnetic Resonance Imaging): Provides soft-tissue detail imaging.
• MIBG Scan (Metaiodobenzylguanidine Scan): A nuclear medicine test to find tumors secreting catecholamines.

3. Genetic Testing

• Certain pheochromocytomas are associated with hereditary syndromes like Multiple Endocrine Neoplasia (MEN) syndrome, Von Hippel-Lindau disease, and neurofibromatosis type 1 (NF1). Genetic testing can be advised for individuals with a family history of these conditions.

Treatment of Pheochromocytoma

The treatment aims to control symptoms and resect the tumor. The plan of treatment varies with the size and extent of the tumor.

1. Medications

Before surgery, medications are given to control symptoms:

• Alpha-blockers (e.g., phenoxybenzamine): Decrease blood pressure by relaxing blood vessels.
• Beta-blockers (e.g., propranolol): Decrease heart rate after alpha-blockers have been given.
• Calcium Channel Blockers (e.g., Nifedipine): Used for blood pressure control.
• Metyrosine decreases catecholamine production in some cases.

2. Surgery for Pheochromocytoma

Surgical excision of the tumor is the definitive treatment.

• Laparoscopic adrenalectomy: A minimally invasive technique utilized for smaller tumors.
• Open Adrenalectomy: This is necessary for larger or malignant tumors.
• Bilateral adrenalectomy: When tumors are found in both adrenal glands.

During surgery, it is critical to monitor the patient closely to avoid blood pressure surges with the release of hormones. Postoperative patients can require lifelong hormone replacement if both adrenal glands are taken out.

Risks and Complications

Despite effective treatment, there are risks and complications, including:

• Fluctuations in blood pressure: These may be preoperative, intraoperative, or postoperative.
• Recurrence: In certain instances, tumors can recur.
• Malignant pheochromocytoma: Rare, but can spread to other organs.
• Adrenal insufficiency: If both adrenal glands are removed, lifelong hormone therapy is required.

Recovery and Prognosis

Most patients recover well after surgery, with significant improvement in blood pressure and other symptoms. Regular follow-ups, blood pressure monitoring, and periodic imaging are necessary to detect any recurrence.

Summery

Pheochromocytoma is a benign but manageable disorder that must be diagnosed and treated quickly. Most patients have a complete recovery after receiving proper treatment, which may include surgery. If you feel high blood pressure, headaches, or profuse sweating, visit a health expert for assessment.