Symptoms of Pheochromocytoma
Pheochromocytoma presents with a classic triad of headaches, palpitations, and sweating occurring in episodic fashion, which has 90% diagnostic specificity when all three symptoms occur together. 1
Hormone-Related Symptoms (Catecholamine Excess)
The hallmark symptoms result from excessive catecholamine release acting on adrenergic receptors throughout the body:
Classic Triad (90% specificity when episodic)
- Headaches - severe and episodic 2, 1
- Palpitations - typically from sinus tachycardia 1, 3
- Sweating - described as "cold sweat" or diaphoresis 1, 4
Cardiovascular Manifestations
- Hypertension - present in approximately 95% of patients, with 50% having sustained hypertension and 50% having paroxysmal (episodic) hypertension 1, 5
- Significant blood pressure variability - an independent risk factor for cardiovascular morbidity and mortality 1, 5
- Tachycardia - from excessive catecholamine stimulation 6, 3
Additional Adrenergic Symptoms
- Pallor - from vasoconstriction 4
- Tremor - from beta-adrenergic stimulation 7
- Anxiety or panic attacks - symptoms closely resemble panic disorder 1, 5, 7
Life-Threatening Presentations
Pheochromocytoma can present with severe cardiovascular complications that may be the first manifestation:
- Hypertensive emergency - from acute catecholamine surge 3
- Myocardial infarction or ischemia - despite normal coronary arteries 7, 6, 3
- Cardiomyopathy - including takotsubo-pattern cardiomyopathy 6, 3
- Cardiac arrhythmias - supraventricular or ventricular tachycardias, less commonly bradycardias or AV blocks 3
- Syncope or cardiac arrest - from severe hemodynamic instability 2
- Stroke or transient ischemic attack - from cerebrovascular effects of catecholamines 3
- Multisystem crisis or shock - from catecholamine storm 3
Non-Hormone-Producing Tumors (Parasympathetic Paragangliomas)
For head and neck paragangliomas that do not produce catecholamines, symptoms arise from mass effect on surrounding structures:
- Hearing loss - from compression of auditory structures 2
- Pulsatile tinnitus - from vascular compression 2
- Cough - from thoracic involvement 2
- Hoarseness - from recurrent laryngeal nerve compression 2
- Dysphagia - from esophageal compression 2
- Facial palsy - from facial nerve involvement 2
- Pain - localized to tumor site 2
- Abnormal tongue motility - from hypoglossal nerve involvement 2
Critical Clinical Pitfalls
- Highly variable presentation - can mimic many other diseases including migraine headaches, panic disorder, cardiac arrhythmias, and myocardial infarction, making diagnosis difficult 8, 7
- Average diagnostic delay of 3 years from initial symptoms to diagnosis 4, 5
- Many cases completely missed - autopsy series show 75% of cases were not suspected during life, with the tumor causing 55% of deaths 5
- Some tumors are clinically silent - presenting only when catecholamine release is triggered by exogenous stimuli such as corticosteroids, procedures, or certain medications 7
When to Suspect Pheochromocytoma
Consider pheochromocytoma in any patient with:
- Paroxysmal hypertension with the classic triad of headaches, palpitations, and sweating 1, 4
- Resistant hypertension - blood pressure >140/90 mmHg despite optimal doses of ≥3 antihypertensive medications including a diuretic 4
- Early-onset hypertension - age <30 years 4
- Apparent panic attacks with concurrent hypertension 1
- Significant blood pressure variability 4
- Family history of pheochromocytoma or hereditary syndromes 4, 5