Imagine experiencing sudden spikes in blood pressure, a racing heartbeat, or intense sweating – symptoms that can feel like an anxiety attack or an urgent medical crisis. For individuals with pheochromocytoma, these episodes are not random; they are caused by a rare tumor on the adrenal glands.
While pheochromocytoma is relatively uncommon, affecting about 2 to 8 people per million annually, its impact on health can be severe if not correctly diagnosed and managed.
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Understanding Pheochromocytoma Attacks
Pheochromocytoma is a rare type of tumor that forms in the adrenal glands, small triangular-shaped glands located on top of each kidney. The adrenal glands are part of the body's endocrine system and secrete hormones that regulate various physiological body functions, including stress responses, heart rate, and blood pressure.
The adrenal gland has two main parts: the outer cortex and the inner medulla.
- The adrenal cortex produces hormones like cortisol, aldosterone, and sex hormones
- The adrenal medulla produces catecholamines, including adrenaline (epinephrine) and noradrenaline (norepinephrine)
Most pheochromocytomas are benign (non-cancerous) and develop in the adrenal medulla (33). They are composed of chromaffin cells, which are responsible for making epinephrine and norepinephrine. These hormones are made during the body's stress ("fight-or-flight") response to prepare it to respond to stressful situations. They increase heart rate, elevate blood pressure, mobilize energy stores, and direct blood flow and oxygen to the muscles.
Pheochromocytomas can push abnormally large amounts of catecholamines into the bloodstream, causing an adrenergic crisis or pheochromocytoma attack. These surges of hormones can occur unpredictably or be triggered by certain factors, including:
- Physical exertion or injury
- Emotional stress
- Childbirth
- Anesthesia and surgery
- Medications, such as certain types of antidepressants (monoamine oxidase inhibitors and tricyclic antidepressants), decongestants, and steroids
- High-tyramine foods, like red wine, chocolate, and cheese
Common Symptoms of Pheochromocytoma Attacks
The most common symptom of pheochromocytoma is high blood pressure (hypertension). More than 60% of people with pheochromocytoma have constant or episodic hypertension, which is often difficult to control and resistant to treatment.
A pheochromocytoma attack can cause a hypertensive crisis, which is when blood pressure reaches dangerously high levels (180/120 mmHg or higher), significantly increasing the risk of organ damage and related symptoms, such as:
- Chest pain
- Shortness of breath
- Nausea and vomiting
- Dizziness
- Confusion
- Changes in vision
- Difficulty speaking
- Loss of consciousness
Other symptoms that patients can experience during an attack include:
- Headache
- Sweating
- Fast and irregular heartbeat
- Shaking and tremor
- Anxiety
- Abdominal pain
- Pale skin
- Diarrhea or constipation
The frequency and severity of pheochromocytomas vary between cases and are often related to tumor size. Some tumors do not make extra adrenaline or noradrenaline, and patients will not experience any symptoms. Others may experience attacks that occur once a month to several times a day. (35, 36)
Diagnosis of Pheochromocytoma
Diagnosing pheochromocytoma can be tricky because it is rare, and its symptoms often mimic other more common conditions like hypertension or anxiety disorders. Because of this, it's often missed or misdiagnosed.
The first step in diagnosing pheochromocytoma is recognizing the typical combination of symptoms, which often occur in sudden, intense episodes.
Once pheochromocytoma is suspected, the next step is to test for elevated levels of catecholamines (adrenaline and noradrenaline) in the blood and urine. One of the following is recommended for initial testing:
- Plasma-Free Metanephrines: This blood test measures the breakdown products of catecholamines (called metanephrines), which are elevated in people with pheochromocytomas.
- 24-Hour Urine Test: Over the course of a day, urine is collected to measure the levels of catecholamines and metanephrines.
If lab results reveal high levels of plasma or urinary metanephrines, the next step is to locate the tumor using imaging scans with either computed tomography (CT) or magnetic resonance imaging (MRI).
Research suggests that up to 35% of pheochromocytomas are linked to inherited genetic conditions. Conditions like multiple endocrine neoplasia type 2 (MEN2), von Hippel-Lindau (VHL) disease, and neurofibromatosis type 1 (NF1) increase the risk of pheochromocytoma. Your doctor may refer you to a genetic counselor for genetic testing to identify mutations in the genes that have been linked to the development of these tumors. (42)
If you are diagnosed with pheochromocytoma, you will need to establish care with an endocrinologist to discuss treatment options. A small percentage (10%) of pheochromocytomas are identified as being malignant (cancerous) based on metastasis (spread) to other parts of the body. If you have a malignant tumor, an oncologist will also need to join your care team.
Management of Pheochromocytoma Attacks
Most pheochromocytomas are unilateral (affecting only one adrenal gland) and benign (22). Surgical removal of the tumor is highly effective, with approximately 90% of patients experiencing resolution of symptoms and normalization of hormone levels after the procedure.
Before surgery, treatment will focus on mitigating symptoms and preventing pheochromocytoma attacks.
Lifestyle Modifications
Trigger avoidance can help reduce the frequency of attacks:
- Diet: Avoid foods that can increase catecholamines in the body. These include tyramine-rich foods (fermented foods, red wine, chocolate, aged meats, and cheese), coffee, tea, bananas, citrus fruits, and vanilla. (42)
- Exercise: Avoid strenuous exercise like heavy lifting, running, or high-intensity workouts, which provokes catecholamine release. Gentle exercises like walking, yoga, and light stretching are safer alternatives until the tumor is managed or removed.
- Stress Management: Emotional stress can activate the body's fight-or-flight response, leading to a surge of catecholamines. Managing stress through relaxation techniques such as meditation, deep breathing exercises, and mindfulness can help lower the risk of triggering an attack.
Medications
Preoperative medication management reduces surgical mortality rates. A preoperative adrenergic blockage is recommended for one to two weeks before surgery to normalize blood pressure and heart rate and prevent a hypertensive crisis during surgery. This is typically achieved with:
Alpha-Blockers
These are the first-line pharmaceutical treatment for managing pheochromocytoma symptoms. Examples include phenoxybenzamine, prazosin, and doxazosin. (17)
These drugs block alpha-adrenergic receptors, to which catecholamines bind to constrict blood vessels. By blocking these receptors, alpha-blockers relax blood vessels and lower blood pressure. (1)
Beta-Blockers
Beta-blockers are used to control rapid heart rate (tachycardia) only after blood pressure has been controlled with alpha-blockers. The use of beta-blockers in the absence of alpha-blockers can lead to hypertensive crisis. Examples include propranolol, metoprolol, and atenolol. (17)
Beta-blockers block beta-adrenergic receptors, which catecholamines use to increase heart rate and contract the heart muscle. By blocking these receptors, beta-blockers help slow the heart rate and reduce the force of the heart's contractions, relieving symptoms like palpitations and tachycardia. (5)
Catecholamine Synthesis Inhibitors
In some cases, a catecholamine synthesis inhibitor, such as metyrosine, is used to reduce the overall production of catecholamines by the tumor. These medications block the enzyme tyrosine hydroxylase, which plays an important role in catecholamine synthesis. (15)
Evidence suggests that metyrosine can reduce urinary metanephrines by 50% and that patients experience better blood pressure control and reduced blood loss during surgery when it is administered with alpha-blockers (15).
Living with Pheochromocytoma
With appropriate treatment, including surgical removal of the tumor, patients with small, benign pheochromocytoma have a five-year survival rate of 95%. Five-year survival rates decrease to 34-60% in recurrent or malignant pheochromocytoma cases. (33)
Pheochromocytoma recurrence rates range between 6.5% and 16.5%, depending on tumor characteristics and the completeness of surgical removal. Therefore, regular follow-up is an important aspect of pheochromocytoma management. After surgery, patients will require ongoing monitoring at least once a year to assess for recurrence or metastasis.
Left untreated, pheochromocytoma poses an increased risk for life-threatening complications. Chronic hypertension can cause damage to the arteries, heart, brain, kidneys, and eyes. It raises the risk of cardiovascular disease and life-threatening events, such as heart attack and stroke.
FAQs on Pheochromocytoma Attacks
1. What are the symptoms of a pheochromocytoma attack?
The most common symptoms of a pheochromocytoma attack include:
- Elevated blood pressure
- Palpitations (rapid or irregular heartbeat)
- Headache
- Sweating
- Feelings of anxiety or panic
2. What triggers a pheochromocytoma attack?
Common triggers for pheochromocytoma attacks include:
- Physical or emotional stress
- Certain medications
- Surgery
- Tyramine-rich foods
3. How are pheochromocytoma attacks different from other hypertensive crises?
Pheochromocytoma attacks are characterized by sudden surges of catecholamines released from the tumor, leading to the rapid onset of symptoms such as palpitations and sweating (34).
In contrast, other hypertensive crises may result from chronic hypertension or underlying health conditions. Most commonly, it is caused by a failure to take prescribed blood pressure medications. (23)
5. What should I do if I experience a pheochromocytoma attack?
If you experience a pheochromocytoma attack, try to stay calm and find a safe place to sit or lie down. Talk to a doctor as soon as possible to discuss these symptoms.
Monitor your blood pressure. If your blood pressure is 180/120 mmHg or higher, retake it after 1-2 minutes. Call 911 immediately if the second reading is just as high and you are experiencing any of the following symptoms:
- Chest pain
- Shortness of breath
- Back pain
- Numbness
- Muscle weakness
- Changes in vision
- Difficulty speaking
- Vomiting
- Dizziness
- Confusion
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Key Takeaways
- Pheochromocytoma is a rare adrenal gland tumor that causes excessive secretion of catecholamines, leading to severe symptoms such as high blood pressure, rapid heart rate, and anxiety – commonly triggered by stress, certain medications, and diet.
- Long-term management of pheochromocytoma attacks is dependent on symptom recognition, timely diagnosis, and surgical intervention.
- Treatment outcomes are improved with preoperative pharmaceutical intervention and lifestyle adjustments to avoid known triggers.
- If you or someone you know is experiencing symptoms associated with pheochromocytoma, don't hesitate to talk to a healthcare provider about your concerns. Early intervention can significantly improve outcomes and enhance quality of life.
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