Pheochromocytoma – Symptoms, Diagnosis, Treatment

Pheochromocytoma is a rare endocrine disease in which there is a production of a large amount of catecholamines (adrenaline and similar hormones) by a tumor. As a result of sudden release of large amounts of adrenaline and noradrenaline, there is a multitude of  symptoms such as heart palpitations, severe headaches, and severe hypertension. However, most of the symptoms are non-specific. That’s why many physicians do not think about pheochromocytoma and it remains un-diagnosed.  That is unfortunate because an untreated pheochromocytpoma can cause sudden death during anesthesia for some unrelated surgery.

Pheochromocytoma tumor often located in the adrenal glands, which are small structures sitting on top of each kidney. However, sometimes this tumor is located outside the adrenal glands somewhere else in the abdomen or even in other parts of the body. The tumor is often benign but rarely it may be malignant.

 In this article, I will discuss symptoms, diagnosis and treatment of Pheochromocytoma.

Symptoms of Pheochromocytoma

There is a long list of symptoms of pheochromocytoma.

Usually symptoms occur in episodes, but may be present all the time.

Attacks of severe headaches, heart palpitations and excessive perspiration in the presence of high blood pressure is the usual combination of symptoms of pheochromocytoma.

Other symptoms include nervousness, anxiety and flushing of skin.

Severe high blood pressure in a young person is another manifestation of pheochromocytoma.

High blood pressure is present in most patients. However, in some patients, blood pressure is normal.

Medical Conditions That Mimic Pheochromocytoma

Many common medical conditions mimic symptoms of pheochromocytoma.

For example, many symptoms of panic attacks – palpitations, nervousness, perspiration – mimic symptoms of pheochromocytoma.

Hyperthyroidism (Overactive Thyroid) is another relatively common condition that has many symptoms similar to  pheochromocytoma symptoms.

Menopause syndrome can give rise to symptoms  – flushing of skin, perspiration, nervousness – which are similar to those of pheochromocytoma.

That’s why it is often difficult to diagnose pheochromocytoma.

A systematic, thorough evaluation by an endocrinologist is the best way to diagnose this rare, but a very important disease.

Tests for Pheochromocytoma

• 24-hour urine collection for metanephrines, nor-metanephrines and free catecholamines
• Blood level of free catecholamines

Localization Of Tumor

Only after the biochemical tests are clearly positive, an endocrinologist does a search for the location of the tumor. The tumor is usually located in one of the adrenal glands. Therefore, a CT scan or an MRI scan of adrenal glands is carried out to locate the tumor. However, sometimes tumor may be located outside the adrenal glands. That’s when things get challenging. Then, more extensive imaging studies have to be done in order to locate the tumor.

Treatment of Pheochromocytoma

Most often treatment is surgical removal of the tumor.

However, medical treatment before, during and soon after surgery is crucial. An experienced endocrinologist, anesthesiologist and surgeon will work together.  Otherwise, serious complications –

and even death – can take place.

Genetic testing:

Sometimes Pheochromocytoma is associated with Medullary thyroid cancer, a rare but deadly thyroid cancer. This is called MEN 2 (Multiple Endocrine Neoplasia 2). It tends to cluster in families. Genetic testing is now available to screen asymptomatic family members of a patient with pheochromocytoma.

In Summary

Pheochromocytoma is a rare endocrine disorder. It has many non-specific symptoms. Seeing an experienced endocrinologist is your best bet for the diagnosis and treatment of pheochromocytoma.

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