Pheochromocytoma – Causes, Symptoms and Treatment
A pheochromocytoma is a rare tumor of the adrenal gland that produces excessive stress hormones, potentially causing dangerous spikes in blood pressure.
Things worth knowing about "Pheochromocytoma"
A pheochromocytoma is a rare tumor of the adrenal gland that produces excessive stress hormones, potentially causing dangerous spikes in blood pressure.
What is a Pheochromocytoma?
A pheochromocytoma is a typically benign tumor that arises from the chromaffin cells of the adrenal medulla. These cells are responsible for producing catecholamines – primarily adrenaline (epinephrine) and noradrenaline (norepinephrine), commonly known as stress hormones. A pheochromocytoma secretes these hormones in an uncontrolled and excessive manner, potentially leading to life-threatening hypertensive crises and a range of other symptoms. Approximately 10% of cases are malignant. In rare instances, similar tumors arise outside the adrenal glands and are referred to as paragangliomas.
Causes
The exact cause of a pheochromocytoma is not always identifiable. Cases are classified as either sporadic or hereditary:
- Sporadic cases: The majority of pheochromocytomas occur without a known genetic cause.
- Genetic syndromes: Approximately 30–40% of pheochromocytomas are associated with hereditary conditions, including Multiple Endocrine Neoplasia type 2 (MEN2), Von Hippel-Lindau disease, Neurofibromatosis type 1, and mutations in SDH genes (succinate dehydrogenase).
Due to the high proportion of hereditary cases, genetic counseling and testing is recommended for all patients diagnosed with this tumor.
Symptoms
The classic symptom triad of pheochromocytoma consists of:
- Episodic or sustained high blood pressure (hypertension)
- Headaches (often severe and throbbing)
- Excessive sweating (diaphoresis)
Other common symptoms include:
- Heart palpitations and rapid heartbeat (tachycardia)
- Facial pallor
- Anxiety and feelings of impending doom
- Tremor
- Nausea and vomiting
- Unexplained weight loss
Symptoms may occur as episodic crises lasting minutes to hours, triggered by physical exertion, stress, certain medications, or direct pressure on the tumor.
Diagnosis
Diagnosing a pheochromocytoma requires a multi-step approach combining biochemical testing and imaging.
Laboratory Tests
The most reliable initial test is the measurement of fractionated metanephrines (catecholamine metabolites) in plasma (blood) or in a 24-hour urine collection. Elevated levels are a strong indicator of a hormone-secreting tumor.
Imaging
Imaging studies are used to locate the tumor:
- CT (computed tomography) or MRI (magnetic resonance imaging) of the abdomen and pelvis for anatomical localization
- MIBG scintigraphy (metaiodobenzylguanidine): a nuclear medicine technique for functional imaging, especially useful in cases of metastases or paragangliomas
- PET-CT using specialized tracers for ambiguous findings
Genetic Testing
Given the high rate of hereditary forms, molecular genetic testing is recommended to identify underlying gene mutations.
Treatment
The treatment of choice for pheochromocytoma is surgical removal of the tumor (adrenalectomy), which is usually performed laparoscopically (minimally invasive). Successful surgery leads to normalization of blood pressure and resolution of symptoms in the majority of patients.
Preoperative Preparation
Medical preparation before surgery is essential to prevent life-threatening blood pressure fluctuations during the procedure. Standard preoperative management includes:
- Alpha-blockers (e.g., phenoxybenzamine or doxazosin) to control blood pressure – initiated 10–14 days before surgery
- Beta-blockers (e.g., propranolol) to manage heart rate – added only after adequate alpha-blockade has been established
Malignant Pheochromocytoma
For malignant or metastatic disease, treatment options include:
- High-dose MIBG therapy (radiolabeled MIBG)
- Chemotherapy (e.g., CVD regimen: cyclophosphamide, vincristine, dacarbazine)
- Targeted therapies (e.g., sunitinib) and investigational agents within clinical trials
Prognosis and Follow-up
The prognosis for benign pheochromocytoma following complete surgical resection is generally favorable. However, there is a risk of recurrence, which is higher in patients with hereditary forms. Regular follow-up is recommended, including biochemical testing (plasma or urine metanephrines) and imaging as needed, for at least 10 years – and lifelong in high-risk patients.
References
- Lenders JW et al. - Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. Journal of Clinical Endocrinology and Metabolism, 2014; 99(6): 1915–1942.
- Neumann HPH, Young WF, Eng C - Pheochromocytoma and paraganglioma. New England Journal of Medicine, 2019; 381(6): 552–565.
- Pacak K, Linehan WM, Eisenhofer G et al. - Recent advances in genetics, diagnosis, localization, and treatment of pheochromocytoma. Annals of Internal Medicine, 2001; 134(4): 315–329.
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