Pheochromocytoma is an interesting type of tumor that arises from the adrenal medulla. It is usually benign (but sometimes it can behave malignant and create metastases), but even despite that it can threaten the patient's life.


The adrenal gland is a pair organ producing a variety of hormones. Adrenal glands are located above the upper pole of both kidneys. The adrenal cortex produces hormones such as mineralocorticoids and glucocorticoids. Adrenal medulla produces hormones known as catecholamines, which can be released into the bloodstream. Adrenaline is probably most known of them all. Adrenaline (and other catecholamines) is released especially during stressful events and they have many effects including increase of the blood pressure and heart rate, constriction of the pupils, dilation of blood vessels in the muscles and narrowing the blood vessels in the skin. The effects should help our body to cope with stressful situations.


The pheochromocytoma is a tumor that arises from medullar cells forming and releasing adrenalin that causes the majority of symptoms.


The exact cause of pheochromocytoma occurence is not known. We assume some influence of a genetic predisposition as pheochromocytoma sometimes occurs as familial disease. There is a link incidence of pheochromocytoma with neurofibromatosis and Von Hippel-Lindau syndrome.


The tumor manifests by paroxysmal secretion of large amount of adrenaline into the bloodstream. The affected person feels effects of adrenalin including elevation of blood pressure, headaches, fast heartbeat with palpitations, sweating, pallor (narrowing of the blood vessels in the skin), anxiety and weight loss. Chronic untreated pheochromocytoma can be fatal due to chronic heart overload resulting in heart failure or due to hemorrhagic stroke.


Note: The pheochromocytoma is often labeled as the 10% tumor. This is because in 10% it is malignant (forms metastases), in 10% it is located outside the adrenal glands, in 10% it occurs in children, in 10% it lacks any classical manifestations, and in 10% it is found simultaneously in both adrenal glands.


The suspicion of the diagnosis can be made due to occurrence of the above-mentioned symptoms. Any case of pheochromocytoma in family history is also of great importance. Te tumor mass may be found by abdominal sonography or computed tomography. Paroxysmal secretion of adrenaline can be confirmed by evaluating concentration of substances of metanephrine or normetanephrine from the urine. These substances are products of adrenaline metabolism and their concentration is often elevated in patients with pheochromocytoma. Nuclear medicine methods or PET CT examination can be used to locate the extra-adrenal tumors.


The best possible solution is the surgical removal of the affected adrenal gland with the tumor. Before the surgical intervention, it is advisable to administer the patient medication dampening effects of adrenaline. When removed, the tumor may excrete a large amount of adrenaline into the patient's blood stream, having potentially fatal consequences.


Jiri Stefanek, MD  Author of texts: Jiri Stefanek, MD
 Sources: basic text sources