Pheochromocytoma

What is pheochromocytoma?

 A pheochromocytoma is a tumor (growth) that arises within the adrenal gland and produces excess hormones (adrenaline & noradrenaline). These high hormone levels can result in dangerously high blood pressure, stroke, heart attacks, kidney failure, and even death if left untreated. Pheochromocytoma is usually benign (non-cancerous) and most are not genetically inherited. Up to 25% of cases are thought to be familial (due to an inherited genetic mutation).

image - pheo pathway

What are the symptoms and signs?

 Patients often present with “spells” of  flushing, heart palpitations, sweating, severe headaches, anxiety, and high blood pressure.  These spells may be triggered by certain medications or stressors. Some patients may experience constipation.  However, some patients may be asymptomatic.

How is pheochromocytoma diagnosed?

 A blood test and/or 24 hour urine collection for the breakdown product of adrenaline & noradrenaline (plasma metanephrines) will be markedly elevated. However, certain medications and disorders (sleep apnea) may falsely elevate results. Once the diagnosis is clear based on blood or urine testing, imaging will be performed (usually MRI or CT) to identify the location of the pheochromocytoma prior to surgery. In certain cases, additional studies such as MIBG or PET scanning may be required. Needle biopsy of the tumor should never be performed to confirm the diagnosis, this can result in hypertensive crisis and potentially death.

Image - pheo diagnosis

Cross-sectional imaging of a large left pheochromocytoma:

Pheo MRI

How is pheochromocytoma treated?

 Initially, your doctor will start you on a blood pressure lowering medication to prevent the potential sequelae of pheochromocytoma (extremely high blood pressure, heart attacks, and stroke). This medication is essential prior to surgery, to avoid blood pressure fluctuations in the operating room when the tumor is manipulated.  Definitive therapy is removal of the tumor (adrenalectomy). Most patients (> 90%) are candidates for minimally invasive surgery (laparoscopic adrenalectomy).

What can I expect after surgery?

Recovery time is usually very quick after minimally invasive surgery, requiring a 1 or 2 night stay in the hospital. Lifelong, your doctor will see you periodically to monitor for recurrence.

Mount Sinai has developed and published a clinical pathway for the comprehensive management of pheochromocytoma.  Read more