Contents
Pheochromocytoma is a neoplasm located in the adrenal glands that develops from pheochromocytomas, and its symptoms are related to the excessive production and release of catecholamines. This condition usually occurs after the age of 40 and may be characterized by palpitations or paroxysmal hypertension.
What is a pheochromocytoma?
Pheochromocytoma is a cancer of the adrenal gland that consists of phaeochromocytoma. The tumor usually occurs in 4-5. decade of life and is closely related to the increased production and release into the blood of large amounts of catecholamines (e.g. adrenaline, dopamine and norepinephrine). These compounds are released mainly under the influence of severe stress and fear, which in turn causes increased heart rate, excessive sweating, pale skin and increased pressure. A tumor may appear in patients suffering from arterial hypertension, but it is only 0,1% of patients.
The causes of the pheochromocytoma
The tumor develops from pheochromocytomas, mostly in the adrenal glands, but it can also appear in places where there are clusters of pheochromocytoma. It is malignant in 10% of patients. May be part of the endocrine neoplasia syndromes.
The tumor can be located in any place where there are clusters of pheochromocytoma, that is:
• in the adrenal medulla,
• in nerve plexuses,
• in the sympathetic ganglia of the nervous system,
• in the very nerves.
However, it is usually found in the abdomen and in the adrenal medulla.
Pheochromocytoma – symptoms
Pheochromocytoma may be asymptomatic, but is more often associated with paroxysmal or permanent hypertension. The symptoms depend on the relationship between the inappropriately produced norepinephrine and adrenaline. The variety of symptoms may be due to hormones (e.g. vasopressin), which may also be released by an adrenal gland tumor. During their ailments, patients complain of:
• headaches,
• palpitations,
• excessive sweating,
• widely dilated pupils,
• muscle tremors,
• feeling anxious,
• pale skin of the face,
• orthostatic hypotension,
• a sharp drop in blood pressure when getting up, for example from a sitting / lying position.
These symptoms are usually paroxysmal and their intensity varies widely. However, some symptoms are related to the action of the following triggers:
• taking medications (e.g. antidepressants, ephedrine, amphetamines, glucocorticosteroids),
• severe stress,
• alcohol consumption,
• physical activity,
• eating heavy meals,
• high temperature,
• compressing the abdomen.
In addition, atypical symptoms may appear that are related to other systems, such as the digestive or cardiovascular systems. Then the patient may experience chest discomfort as well as nausea and abdominal pain.
Complications of pheochromocytoma
There may be a shock and so-called «Sharp abdomen» due to rupture of the tumor and hence bleeding into the abdominal cavity. On the other hand, sudden changes in blood pressure that occur during surgery under general anesthesia are a direct threat to life.
Important! In pregnant women, phaeochromocytoma can lead to miscarriage!
Pheochromocytoma – diagnosis The tests used in the diagnosis of pheochromocytoma of the adrenal glands include:
• imaging examination that reveals where the tumor is located (computed tomography, ultrasound, MRI); sometimes adrenal scintigraphy or positron emission tomography are also performed;
• laboratory tests that reveal catecholamine metabolites in urine and serum;
• histopathology – is needed to determine the degree of tumor malignancy and confirm suspicions of a tumor;
• genetic test – performed when the phaeochromocytoma is present in family,
• tests stimulating the tumor to release hormones – are used in some cases (with the use of glucagon),
• tests with substances that inhibit the secretion of catecholamines.
Treatment of a pheochromocytoma of the adrenal gland
Surgical treatment is considered the basic method. Usually, laparoscopy is performed, which is less invasive, but sometimes the decision is made on the classic method – i.e. opening the abdominal cavity. Surgical procedures for pheochromocytoma are quite complicated and the patient should be very well prepared for them. The most important thing is to give the patient before the procedure measures to prevent dangerous sudden pressure surges during the surgery. These are usually alpha and beta blood pressure blockers. In addition, remember to balance the patient’s water and electrolyte balance.
After surgery, patients should be monitored for blood pressure and the presence of catecholamines in blood and urine for several days. Usually, surgery improves and alleviates clinical symptoms and normalizes blood pressure. However, there are cases where, despite surgery, there has been no improvement. This may be related to a family history of pheochromocytoma or its location outside the adrenal glands.
What’s the prognosis?
After surgery, the prognosis is very good, but only if the tumor was not malignant and the tumor was not located outside the adrenal glands. If the cancer is related to the family history in a given patient, the prognosis is much worse.
Lit.: [1] Manger W.M.: An overview of pheochromocytoma: history, current concepts, vagaries, and diagnostic challenges. Ann N Y Acad Sci 2006, 1073; 1-20. [2] Heymann W.R.: Flushing, pheochromocytoma, and the dermatologist. J Am Acad Dermatol 2006, 55; 1075-7.
Source: A. Kaszuba, Z. Adamski: “Lexicon of dermatology”; XNUMXst edition, Czelej Publishing House