What is pheochromocytoma
Pheochromycytoma is a tumor that develops in the adrenal gland, generally affecting its innermost portion, called the medulla, where the chromaffin cells are located. 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 the intermediate age groups.
Adrenals and Catecholamines
The adrenal glands, placed like a hat above the kidneys, produce two very important hormones in their central portion, 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 reaction of attack and flight. In similar circumstances, thanks to the massive secretion of these hormones, the heart increases the force and the contractile frequency, the bronchi, the pupil and the blood vessels of the appendicular muscles and the coronary system dilate, while glycogenolysis is stimulated in the liver.
At the same time, always in order to prepare the body for the imminent physical activity, the digestive processes are significantly slowed down, while the cutaneous and peripheral blood vessels constrict and the arterial pressure increases; the bladder relaxes, while the urethral sphincter narrows (inhibits urination).
Symptoms
For further information: Pheochromocytoma Symptoms
Since pheochromocytoma is a catecholamine-secreting tumor, it is very often accompanied by hypertension (a fundamental clinical manifestation) and symptoms such as sudden headache, pallor, profuse sweating, chest pain, weight loss, nervousness, anguish, anxiety, tremors, palpitations and sensation of more energetic heartbeat. Sometimes it can be asymptomatic.
Sometimes the symptoms of pheochromycotoma also include gastrointestinal disorders, such as abdominal pain, nausea and vomiting, and impaired glucose metabolism, such as impaired glucose tolerance or overt diabetes.
In relation to the release of catecholamines by the tumor, the symptoms can occur in an accessory way (sudden and violent attacks, lasting less than an hour) or continuous. target organs (e.g. retinal problems).
Symptoms of pheochromocytoma can suddenly worsen resulting in hypertensive crises; this occurs mainly as a result of emotional stress or anxiety, surgical anesthesia or physical exertion that cause a certain pressure on the tumor (changes in position, sports, diagnostic maneuvers (catheterization), lifting a weight, defecation, urination, pregnancy, etc.) . Symptoms of pheomocrocytoma can also be exacerbated by the use of certain drugs, drugs (amphetamines, high-dose caffeine, ephedrine, decongestants, antihistamines, cocaine, etc.) and foods (those rich in tyramine), which raise blood pressure. presence of pheochromictoma should also be avoided the use of metoclopramide, atropine and MAO-inhibitor drugs (risk of hypertensive crisis with heart damage); among the latter we remember isocarboxazide, phenelzine, selegiline, moclobemide and tranylcypromine. Hypertensive crises can be controlled with drugs such as nitroglycerin and derivatives.
Diagnosis
See also: Urinary Metanephrines
The diagnosis of pheochromocytoma is based on the dosage of adrenaline and noradrenaline, and their metabolites (metanephrines), in the blood and urine. If levels are elevated, the exact location of the pheochromocytoma is identified by CT, abdominal MRI, 6- (18F) fluorodopamine PET scan, or by meta-iodobenzylguanidine (MIBG) or somatostatin analogue scintigraphy. (octreoscan).
Treatment
The treatment of choice, when practicable, is the surgical one.
With the removal of the adrenal gland affected by pheochromocytoma, the symptoms tend to vanish in most cases; heart pressure also returns to normal. If the neoplasms affect both glands, both adrenal glands must therefore be removed; unfortunately bilateral adrenalectomy requires chronic replacement therapy to replace various adrenal hormones, such as cortisol and aldosterone.
Whenever possible, surgery is performed laparoscopically, ie by inserting fine precision instruments into small incisions made in the patient's abdomen.
It is essential, while waiting for the surgery and in preparation for it, to take alpha and beta blocking drugs and, if necessary, other drugs to control blood pressure and the various symptoms triggered by pheochromocytoma.
