How pheochromocytoma is suspected?

In the present times and for the majority of patients, pheochromocytoma becomes a concern in 3 situations: symptoms, adrenal tumor, and familial syndromes.

The symptoms of pheochromocytoma are very diverse thus pheochromocytoma has earned the name as a "master mimicker" of other diseases. The symptoms can be as simple as mild hypertension but can also be as dramatic as strokes. The unique symptoms of pheochromocytoma "spells" are not as common nowadays as described in the classic literature. The spells may be elicited by emotional stress, exertion, pressure to the abdomen, or other stimuli the patients themselves often can figure out and avoid. The spells start with a sudden sense of doom or extreme fear, accompanied with headache, heart pounding, sweating, eye sensitivity to light, and other symptoms. A spell can last a few minutes to much longer. Blood pressure is invariably increased initially during a spell but blood pressure may drop to hypotension range due to reflexes of the body. Unusual hypertension and one or more components of the spell may make the doctor suspect pheochromocytoma. Only 0.2%-2% people with these symptoms actually have pheochromocytoma while symptoms are caused by other diseases in the majority of patients.

A mass in the adrenal glands is another reason for the doctor to suspect pheochromocytoma. Up to 5% of adrenal masses are pheochromocytoma. Therefore, tests for pheochromocytoma should be performed whenever a patient is found to have an adrenal mass. Pheochromocytoma found this way can be "silent" and the patient may be even free of symptoms.

The third situation is monitoring people who are carriers for mutations that cause familial tumor syndromes. Multiple endocrine neoplasia type II (MENII), von Hippel-Lindau disease (VHL), neurofibromatosis type 1 (NF1), and familial paraganglioma syndrome are the most common syndromes. Carriers of the mutations related to these syndromes need to be screened periodically by blood tests and imaging.

Of course a combination of any of the 3 situations can occur.