Pheochromocytoma • tumor of the adrenal medulla

High blood pressure is a widespread disease and is often the result of an unhealthy lifestyle: In a few rare cases, however, the cause is a pheochromocytoma. This is a tumor that produces large amounts of hormones and thus increases blood pressure. You can read here what other symptoms are typical, how the disease is treated and what the chances of recovery are.

A pheochromocytoma is a tumor of the adrenal medulla and the cells produce hormones. This leads to an excess of hormones. High blood pressure is a typical symptom of the disease.

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What is a pheochromocytoma?

A pheochromocytoma is a hormone-producing tumor of the adrenal medulla. Sometimes the tumor also grows along the spine on the so-called sympathetic trunk, then experts also speak of an extraadrenal pheochromocytoma or paraganglioma. If the tumor is located in the adrenal medulla, around 85 percent of the tumors are benign and 15 percent are malignant; in the case of a paraganglioma, around a third of the tumors are malignant.

As a rule, the disease occurs unilaterally; the pheochromocytoma only grows on both halves of the body in around ten percent of cases.

What hormones produce a pheochromocytoma?

About two thirds of the pheochromocytomas that occur form the stress hormones noradrenaline and adrenaline, in paragagliomas above the diaphragm only noradrenaline is produced. Malignant pheochromocytomas also release dopamine.

Causes and risk factors: Often hereditary

A pheochromocytoma is one of the rare diseases, annually only about two to eight out of a million people develop the hormone-producing tumor. The causes are unclear in about two thirds of cases, but up to 30 percent have a genetic cause and are related to hereditary diseases, including:

  • Neurofibromatosis type 1 (Recklinghausen’s disease)
  • Multiple Endocrine Neoplasia Type 2 (MEN 2 Syndrome)
  • Von Hippel-Lindau Syndrome

In those affected who are younger than 40 years of age, and especially in children, there is usually a corresponding genetic cause.

Pheochromocytoma: symptoms often ambiguous

The symptoms caused by a pheochromocytoma are often rather diffuse and can occur in many diseases. They are mainly due to the increased levels of hormones in the body.

Adrenaline and noradrenaline are hormones; they act as neurotransmitters in the autonomic nervous system, regulate body functions and are released primarily in response to stress. For example, they ensure better blood circulation, regulate pulse and blood pressure and also have an effect on digestion.

If too much of it is formed over the long term, problems arise in the body. For example, high blood pressure is a result of pheochromocytoma. A distinction is made between two types of hypertension, which are triggered by the tumor.

With paroxymal hypertension, blood pressure rises suddenly, it can lead to a hypertensive crisis, which is a medical emergency. In addition, there can also be a permanent increase in blood pressure (persistent hypertension). In adults, the paroxymal form occurs in half of the cases, in the other half of those affected the blood pressure is permanently elevated. In children, on the other hand, the seizure-like increase in blood pressure of ten percent is the much rarer variant.

In addition, other symptoms can occur in the course of the disease:

In addition, diabetes mellitus can develop as a result of the permanently changed hormone level.

Diagnosis: how is a pheochromocytoma diagnosed?

In very few cases the disease is symptom-free. If the tumor is discovered by chance using certain imaging techniques, a laboratory test can confirm the diagnosis. If a pheochromocytoma is suspected, certain metabolic products (so-called metanephrines and normetanephrines) of adrenaline, noradrenaline and dopamine are determined from the 24-hour urine collection. The direct determination from the blood plasma, however, is not advisable, because they vary a lot even in healthy people.

As a rule, and especially if the laboratory values ​​are slightly elevated, a second test is required, but a drug (clonidine) is administered beforehand. The clonidine test gives certainty: In healthy people, the production of hormones is inhibited, but if there is a pheochromocytoma, the values ​​are still elevated.

Even if the suspected diagnosis is made based on the symptoms, a laboratory test is usually carried out.

Imaging techniques help make the diagnosis. Depending on the location, various examination methods can detect the tumor:

  • In the adrenal medulla: Tumors in the adrenal medulla can be visualized using computed tomography (CT) and magnetic resonance imaging (MRI)

  • Paragangliom (extraadrenales Paragangliom): Here, other imaging methods must be used, such as MIBG scintigraphy (nuclear medicine method) or the combined DOPA-PET-CT (positron emission tomography combined with CT, in which a precursor of dopamine is enriched in the tumor cells, which causes it becomes visible)

How is a pheochromocytoma treated?

Treatment consists primarily of removing the hormone-producing tumor tissue. As far as possible, the procedure is laparoscopic (minimally invasive). In most cases, no major surgery is necessary; small incisions are sufficient. If the tumor is particularly large or poorly located, a major intervention and the opening of the abdominal wall may be necessary. Before the surgical or laparoscopic removal, antihypertensive drugs are usually administered about one to two weeks beforehand so that there is no hypertensive emergency during the procedure.

In other rare cases, the tumor is inoperable and cannot be removed or cannot be removed completely, in which case a drug treatment that inhibits the formation of adrenaline and noradrenaline is indicated. Alpha blockers are often used.

If it is a malignant pheochromocytoma (cancer) that can form metastases, chemotherapy or radioiodine therapy is sometimes necessary in addition to the surgical removal of the tumor tissue. Lightly radioactive iodine is administered as an injection (as meta-iodine-benzylguanidine (MIBG)), which is absorbed by the tumor cells. The cancer is then locally irradiated.

Every treatment is followed by follow-up examinations. The disease recurs in around 15 percent of those affected.

Prognosis: What is the life expectancy with a pheochromocytoma

The prognosis for a pheochromocytoma depends on many factors. If the tumor is detected early, it is benign and if it is located in such a way that it can be easily removed, the chances of recovery are good.

If diagnosed later, the high blood pressure caused can damage the body and attack blood vessels. Diabetes mellitus can also be the result if the pheochromocytoma is detected late and not treated.

In the case of a malignant pheochromocytoma, the chances of a cure drop drastically, especially if the cancer has already spread to other organs and metastases in the liver, for example. Therefore, early detection and treatment is of great importance here too.

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Source: Lifeline | Das Gesundheitsportal by www.lifeline.de.

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