Pheochromocytoma is a rare tumor that develops in the adrenal glands, typically in the center portion called the adrenal medulla. These tumors produce excessive amounts of catecholamines, which are hormones like adrenaline and noradrenaline that regulate heart rate, blood pressure, and metabolism. While pheochromocytomas are usually benign (non-cancerous), they can cause serious health complications if left untreated due to the excess hormone production.
Recognizing the symptoms of pheochromocytoma is crucial for early diagnosis and appropriate management. The symptoms can vary widely from person to person and may occur in episodes or attacks that last from a few minutes to several hours. Understanding these warning signs can help you seek timely medical attention and potentially prevent life-threatening complications.
1. Severe High Blood Pressure (Hypertension)
One of the hallmark symptoms of pheochromocytoma is dramatically elevated blood pressure. This hypertension can manifest in two distinct patterns: sustained high blood pressure that remains consistently elevated, or paroxysmal hypertension characterized by sudden, severe spikes in blood pressure.
During hypertensive episodes, blood pressure readings can soar to dangerously high levels, sometimes exceeding 200/120 mmHg. These episodes may occur spontaneously or be triggered by specific factors such as physical exertion, emotional stress, certain foods, or even simple activities like bending over or urinating. The excessive release of catecholamines causes blood vessels to constrict, leading to these dramatic increases in blood pressure.
Key characteristics:
- Sudden onset of extremely high blood pressure
- Blood pressure that doesn’t respond well to standard antihypertensive medications
- Readings that may fluctuate dramatically throughout the day
- Young patients with unexplained hypertension
2. Severe Headaches
Intense, throbbing headaches are among the most common and distressing symptoms experienced by people with pheochromocytoma. These headaches are typically described as severe, sudden in onset, and distinctly different from ordinary headaches or migraines.
The headaches result from the sudden surge in blood pressure caused by catecholamine release. They often feel like intense pressure or pounding sensation, usually affecting the entire head or concentrated in the temples and back of the head. These headaches may be accompanied by a feeling of pressure behind the eyes and can be so severe that they interfere with daily activities.
Unlike typical tension headaches, pheochromocytoma-related headaches often occur in conjunction with other symptoms like sweating and rapid heartbeat, forming what doctors call the “classic triad” of pheochromocytoma symptoms.
3. Excessive Sweating (Hyperhidrosis)
Profuse sweating, often described as drenching or soaking, is another cardinal symptom of pheochromocytoma. This sweating is typically excessive and disproportionate to the ambient temperature or level of physical activity.
Patients often report sudden episodes of intense sweating that can soak through clothing and bedding, occurring during both day and night. The sweating may affect the entire body or be particularly pronounced on the face, neck, and upper body. This symptom occurs because catecholamines affect the sweat glands and metabolic rate, causing the body to produce excessive amounts of perspiration.
The sweating episodes often coincide with spikes in blood pressure and heart rate, and may be accompanied by feelings of heat or flushing. Some patients experience cold, clammy sweats rather than warm perspiration.
4. Rapid or Irregular Heartbeat (Palpitations)
Heart palpitations are a frequent and often frightening symptom of pheochromocytoma. Patients describe feeling their heart racing, pounding, or beating irregularly, sometimes feeling like the heart is “skipping beats” or “fluttering” in the chest.
The excessive catecholamines released by the tumor directly stimulate the heart, causing it to beat faster and more forcefully. Heart rates during episodes can exceed 100-120 beats per minute, even when at rest. Some patients may also experience arrhythmias, where the heart beats in an irregular pattern.
Associated cardiac symptoms may include:
- Sensation of heart pounding in the chest, neck, or throat
- Rapid heartbeat (tachycardia) that occurs suddenly
- Chest discomfort or tightness
- Feeling of anxiety or impending doom accompanying the palpitations
5. Tremors and Shaking
Trembling or shaking, particularly of the hands, is a common symptom caused by the excess adrenaline circulating in the body. These tremors can range from mild, barely noticeable shaking to pronounced trembling that interferes with fine motor tasks like writing, holding objects, or using utensils.
The tremors typically worsen during catecholamine surges and may be accompanied by an internal feeling of jitteriness or restlessness, similar to the sensation of having consumed too much caffeine. Some patients describe feeling “shaky inside” even when external tremors aren’t visible to others.
These tremors result from the direct effect of catecholamines on muscles and the nervous system, causing increased muscle tension and involuntary muscle contractions.
6. Pale Skin (Pallor)
Sudden paleness or a pale, ashen appearance of the skin is another notable symptom of pheochromocytoma. This pallor occurs because the excess catecholamines cause blood vessels in the skin to constrict, reducing blood flow to the skin’s surface.
During episodes, patients may notice their face appears unusually pale or white, and their skin may feel cool to the touch despite excessive sweating. This symptom is particularly noticeable in individuals with lighter skin tones but can occur in anyone. The pallor may alternate with flushing (redness) as the episode progresses and blood vessels subsequently dilate.
Family members or caregivers often notice this change in skin color before the patient themselves, as it can be quite dramatic and sudden in onset.
7. Anxiety and Sense of Impending Doom
Many patients with pheochromocytoma experience intense feelings of anxiety, nervousness, or a sense of impending doom, particularly during catecholamine surges. These psychological symptoms can be just as distressing as the physical manifestations.
The anxiety isn’t simply a reaction to the physical symptoms; rather, it’s a direct result of the excess adrenaline affecting the brain and nervous system. Patients describe feeling intensely anxious, panicked, or fearful without any obvious trigger. Some report feeling like something terrible is about to happen, even when they’re in safe, familiar environments.
Psychological symptoms may include:
- Sudden panic attacks
- Overwhelming sense of dread or fear
- Restlessness and inability to relax
- Difficulty concentrating
- Irritability or mood changes
These symptoms can sometimes lead to misdiagnosis as panic disorder or anxiety disorders, especially when they occur without obvious physical triggers.
8. Chest or Abdominal Pain
Pain in the chest or abdomen is a significant symptom that should never be ignored. Chest pain in pheochromocytoma can result from the increased workload on the heart, coronary artery spasms caused by catecholamines, or in severe cases, stress on the heart muscle (cardiomyopathy).
The chest pain may feel like pressure, tightness, or squeezing in the chest and can sometimes mimic the symptoms of a heart attack. This is why any chest pain requires immediate medical evaluation to rule out cardiac emergencies.
Abdominal pain, on the other hand, may be related to the tumor’s location in the adrenal glands or can result from intestinal effects of excess catecholamines. Some patients describe a sense of fullness, pressure, or aching in the upper abdomen or flank area where the adrenal glands are located.
9. Unexplained Weight Loss
Significant weight loss without intentional dieting or increased exercise is another important symptom of pheochromocytoma. This occurs because the excess catecholamines dramatically increase the body’s metabolic rate, causing it to burn calories at an accelerated pace.
Patients may notice they’re losing weight despite maintaining their normal eating habits, or in some cases, eating more than usual. The weight loss can be gradual or rapid, and may be accompanied by other metabolic symptoms such as increased appetite, heat intolerance, and fatigue.
The elevated metabolism caused by continuous catecholamine excess puts the body in a constant “fight or flight” state, consuming energy reserves and leading to progressive weight loss over time.
10. Nausea and Vomiting
Gastrointestinal symptoms, particularly nausea and vomiting, can occur during catecholamine surges in pheochromocytoma. These symptoms result from the effects of excess hormones on the digestive system, which can slow gastric emptying and affect intestinal motility.
Some patients experience chronic nausea that worsens during episodes of hormone excess, while others have sudden bouts of nausea and vomiting during acute attacks. Additional gastrointestinal symptoms may include constipation, as catecholamines can slow down bowel movements, or occasionally diarrhea.
The nausea may be severe enough to interfere with eating and can contribute to the weight loss commonly seen in this condition. Some patients also report a general feeling of abdominal discomfort or changes in bowel habits.
Main Causes of Pheochromocytoma
Understanding the causes of pheochromocytoma can help identify individuals at higher risk for developing this rare tumor. While the exact cause isn’t always clear, several factors have been identified:
Genetic Factors
Hereditary syndromes: Approximately 30-40% of pheochromocytomas are associated with inherited genetic conditions. Several hereditary syndromes significantly increase the risk of developing these tumors:
- Multiple Endocrine Neoplasia type 2 (MEN2): An inherited condition that causes tumors in multiple endocrine glands, including the adrenal medulla
- Von Hippel-Lindau (VHL) disease: A genetic disorder that causes tumors and cysts throughout the body, including pheochromocytomas
- Neurofibromatosis type 1 (NF1): A condition that causes tumors to form on nerve tissue
- Hereditary paraganglioma-pheochromocytoma syndromes: Caused by mutations in genes such as SDHB, SDHD, SDHC, and SDHA
Sporadic Cases
The majority of pheochromocytomas (60-70%) occur sporadically without a clear hereditary pattern. In these cases, the tumor develops due to random genetic mutations that occur during a person’s lifetime rather than being inherited. The triggers for these spontaneous mutations are not fully understood.
Age and Demographics
While pheochromocytomas can occur at any age, they are most commonly diagnosed in adults between 30 and 50 years old. However, hereditary forms tend to appear at younger ages. The condition affects men and women roughly equally.
Other Risk Factors
Having a family history of pheochromocytoma or related genetic syndromes increases the risk significantly. If one or more family members have been diagnosed with this tumor or associated genetic conditions, genetic counseling and testing may be recommended.
Frequently Asked Questions (FAQ)
How rare is pheochromocytoma?
Pheochromocytoma is quite rare, occurring in approximately 2-8 per million people annually. It accounts for less than 0.2% of cases of high blood pressure. Despite its rarity, it’s an important condition to diagnose because it’s a potentially curable cause of hypertension.
Can pheochromocytoma symptoms come and go?
Yes, symptoms of pheochromocytoma are often episodic, meaning they come and go in attacks or spells. These episodes can last from a few minutes to several hours and may occur multiple times per day or only occasionally. Between episodes, some patients feel completely normal, while others have persistent symptoms. The unpredictable nature of symptoms can make diagnosis challenging.
What triggers a pheochromocytoma attack?
Various factors can trigger symptom episodes in pheochromocytoma, including physical activities like exercise or bending over, emotional stress or anxiety, certain foods (especially those containing tyramine like aged cheese and cured meats), caffeine and alcohol, certain medications (including some anesthetics and decongestants), changes in body position, pressure on the abdomen, and urination. However, episodes can also occur spontaneously without any identifiable trigger.
Is pheochromocytoma dangerous if left untreated?
Yes, untreated pheochromocytoma can be life-threatening. The persistently high levels of catecholamines can lead to serious complications including stroke, heart attack, heart rhythm abnormalities, heart failure, kidney damage, and vision problems from damaged blood vessels in the eyes. During severe episodes, extremely high blood pressure can cause hypertensive crisis, which is a medical emergency. This is why prompt diagnosis and appropriate management are essential.
How is pheochromocytoma diagnosed?
Diagnosis typically involves a combination of blood and urine tests to measure catecholamine levels and their metabolites (metanephrines), imaging studies such as CT scans, MRI, or specialized nuclear medicine scans to locate the tumor, and genetic testing in some cases, especially for younger patients or those with family history. Your doctor may also monitor blood pressure patterns and review your symptom history carefully.
Can pheochromocytoma occur in children?
While less common, pheochromocytoma can occur in children and adolescents. In pediatric cases, there’s a higher likelihood of the tumor being hereditary, with up to 70% of childhood cases associated with genetic syndromes. Children may present with similar symptoms to adults, including high blood pressure, headaches, sweating, and palpitations. Any child with unexplained hypertension should be evaluated for possible pheochromocytoma.
Are all pheochromocytomas cancerous?
No, the vast majority of pheochromocytomas (approximately 90%) are benign, meaning they are not cancerous. However, about 10% can be malignant (cancerous) and have the potential to spread to other parts of the body. The term “malignant pheochromocytoma” is typically reserved for tumors that have metastasized (spread) to sites where chromaffin tissue is not normally present, such as bones, liver, lungs, or lymph nodes.
Should I see a doctor if I have these symptoms?
Absolutely. If you experience the combination of severe headaches, excessive sweating, and rapid heartbeat, especially along with high blood pressure, you should seek medical evaluation promptly. While these symptoms can be caused by many conditions, it’s important to rule out pheochromocytoma. If you have a family history of pheochromocytoma or related genetic syndromes, inform your healthcare provider, as you may need screening even without symptoms.
Can stress cause pheochromocytoma?
No, stress does not cause pheochromocytoma. The tumor develops due to genetic factors, either inherited or acquired mutations. However, emotional stress can trigger symptom episodes in people who already have pheochromocytoma, causing the tumor to release more catecholamines and precipitating an attack. This is why symptoms are sometimes mistaken for panic attacks or stress-related conditions.
References:
- Mayo Clinic – Pheochromocytoma
- National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
- National Center for Biotechnology Information (NCBI) – Pheochromocytoma
- Johns Hopkins Medicine – Pheochromocytoma
- Endocrine Society
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