Primary Pheochromocytomas

Primary pheochromocytomas (PCs) are rare catecholamine-secreting tumors derived from chromaffin cells of the autonomic nervous system that develop in the period between birth and the third decade of life. PCs usually arise within the adrenal glands, but they may rarely occur in extra-adrenal sites such as the urinary bladder. The diagnosis of pheochromocytoma is based on biochemical and genetic tests and imaging studies.

Pheochromocytomas are mainly treated surgically by removal of the affected adrenal gland or resection of the extra-adrenal tumor. If there is metastatic disease, medical treatment with antineoplastic agents may be indicated.

Signs and Symptoms

Signs and symptoms of primary pheochromocytoma vary from person to person and may include:

Severe headache
Sweating
Palpitations
High blood pressure
Anxiety
Chest pain
Abdominal pain
Paroxysmal hypertension

In more serious cases, symptoms may also include:

Hypertensive crisis
Paleness
Fainting
Weight loss
Labile hypertension

Diagnosis

The diagnosis of primary pheochromocytoma is based on imaging studies, such as computed tomography (CT) scan or magnetic resonance imaging (MRI) scan. Biochemical tests, including measurement of catecholamine levels (such as epinephrine, norepinephrine, and dopamine) in the blood, urine, or saliva, as well as genetic tests, can also be used to diagnose primary pheochromocytoma.

Treatment

The treatment of primary pheochromocytoma usually involves surgical removal of the tumor. If the tumor is in the adrenal gland, the entire gland may be removed. If the tumor is in an extra-adrenal site, it may be removed without removing the entire organ.

If the tumor is not amenable to surgical removal, medical treatment with antineoplastic agents may be indicated. In addition, the use of alpha-blockers, beta-blockers, and other medications may be necessary to control the symptoms of pheochromocytoma.