Pheochromocytoma

What is pheochromocytoma

Pheochromicitoma is a tumor that develops in the adrenal gland, generally affecting its innermost portion, called medulla, where chromaffin cells are found. It is a rather rare neoplasm, with an estimated incidence between 2 and 8 cases per million inhabitants; pheochromocytoma recognizes a certain family predisposition and is more common among young adults and in middle age groups.

Normally the pheochromocitroma is unique and affects only one adrenal gland, but there are exceptions. Since chromaffin cells are also found in nervous tissue, pheochromocytomas can also have extra-adrenal localization, thus arising outside of these glands. In these cases we prefer to talk about paragangliomas, neoplasms that can affect the heart, the neck, the bladder or the posterior wall of the abdomen. Pheochromocytoma is generally benign (90% of cases), but reversing the normal function of the adrenal gland causes a series of rather important symptoms.

Adrenal glands and catecholamines

The adrenal glands, placed like a hat over the kidneys, produce in their central portion two very important hormones, respectively called adrenaline and noradrenaline, or more generally catecholamines. Together, they regulate the body's response to psychophysical stress, preparing it for the so-called attack and flight reaction. In such circumstances, thanks to the massive secretion of these hormones, the heart increases the strength and the contractile frequency, the bronchi, the pupil and the blood vessels of the appendicular and coronary system dilate, while at the liver level the glycogenolysis is stimulated.

At the same time, again with the aim of preparing the body for impending physical activity, the digestive processes are significantly slowed down, while the cutaneous and peripheral blood vessels become constricted and the arterial pressure increases; the bladder relaxes, while the urethral sphincter narrows (inhibits urination).

Symptoms

To learn more: Pheochromocytoma symptoms

Since pheochromocytoma is a catecholamine-secreting tumor, it is very often accompanied by hypertension (fundamental clinical manifestation) and symptoms such as sudden headaches, pallor, profuse sweating, chest pain, weight loss, nervousness, anxiety, anxiety, tremors, palpitations and sensation of more energetic heart beat. Sometimes it can be asymptomatic.

Sometimes the symptoms of pheochromicotoma include gastrointestinal disorders, such as abdominal pain, nausea and vomiting, and alterations of glucose metabolism, such as reduced glucose tolerance or a diabetes free.

In relation to the release of catecholamines from the tumor, the symptomatology can occur in an accessory way (sudden and violent attacks, lasting less than an hour) or continuous. Furthermore, the consequences of arterial hypertension on target organs (eg retinal problems) can be added to all these symptoms.

The symptoms of pheochromocytoma can suddenly worsen giving rise to hypertensive crises; this happens above all following emotional stress or anxiety, surgical anesthesia or physical efforts that cause a certain pressure on the tumor (changes in position, sporting activity, diagnostic maneuvers (catheterization), lifting of a weight, defecation, urination, pregnancy, etc.) . The symptoms of the pheomocrocytoma can also be exacerbated by the use of some drugs, drugs (amphetamines, high-dose caffeine, ephedrine, decongestants, antihistamines, cocaine, etc.) and foods (those rich in tyramine), which raise blood pressure. The use of metoclopramide, atropine and MAO inhibitory drugs (risk of hypertensive crises with cardiac damage) should also be avoided in the presence of pheochromycoma; among the latter we mention isocarbossazide, phenelzine, selegiline, moclobemide and tranylcypromine. Hypertensive crises can be controlled with drugs such as nitroglycerin and derivatives.

Diagnosis

Treatment

The treatment of choice, when practicable, is surgical.

In fact, with the removal of the adrenal gland affected by the pheochromocytoma, the symptoms tend to disappear in most cases; cardiac pressure also returns to normal. If the neoplasms involve both glands, it is therefore necessary to remove both adrenals; unfortunately, bilateral adrenalectomy requires chronic replacement therapy to replace the various adrenal hormones, such as cortisol and aldosterone.

When possible, surgery is performed laparoscopically, that is by inserting thin precision instruments into small incisions made in the patient's abdomen.

Indispensable, pending the intervention and in preparation for it, the intake of alpha and beta blockers and, if necessary, of other drugs to control blood pressure and the various symptoms triggered by pheochromocytoma.