What is Pheochromocytoma?
A pheochromocytoma is a rare tumor that typically develops in the medulla (inner part) of the adrenal gland. A pheochromocytoma is a tumor originating from the chromaffin tissue (sympathetic) of the adrenal medulla. A paraganglioma, on the other hand, is a tumor of chromaffin cells from the sympathetic or parasympathetic chain, located outside the adrenal gland. Parasympathetic paragangliomas rarely (less than 5%) lead to clinical symptoms by obvious catecholamine synthesis. The WHO-2017 classification states that the histological findings in these two tumor types are similar, but a pheochromocytoma is an adrenal tumor and a paraganglioma is an extra-adrenal tumor. This tumor leads to an excessive secretion of catecholamines, known as stress hormones such as adrenaline (epinephrine) and noradrenaline (norepinephrine). This increase in hormone levels is characterized by sudden and uncontrolled blood pressure spikes, palpitations, sweating, and headaches. A pheochromocytoma is mostly benign, but in some cases, it can have the potential for malignancy.
What Are The Types Of Pheochromocytoma?
Pheochromocytomas can be classified into different subtypes based on their location and genetic basis. Approximately 30-40% have a hereditary component, which necessitates genetic testing for all patients. The most common form is adrenal (adrenal gland-associated) pheochromocytoma. Additionally, extra-adrenal tumors called paragangliomas, which can develop along the sympathetic nervous system, can also cause a similar clinical picture. Forms associated with hereditary syndromes include Multiple Endocrine Neoplasia type 2 (MEN2), Von Hippel-Lindau disease, and Neurofibromatosis type 1. In these types, the disease generally appears at a younger age and is associated with bilateral adrenal gland involvement or additional tumors.
What Are The Symptoms And Diagnosis Methods For Pheochromocytoma?
One of the most prominent complaints in patients with pheochromocytoma is paroxysmal (episodic) hypertension. This can be accompanied by symptoms such as palpitations, excessive sweating, headaches, feelings of anxiety, tremors, facial flushing, and weight loss. These symptoms can be triggered especially by physical exertion, stress, anesthesia, surgical procedures, or exposure to certain foods and medications.
In the diagnostic process, the comprehensive evaluation performed by Uzm. Dr. Burcu Meryem Atak Sançmış is critically important. Measuring plasma levels of free metanephrines and normetanephrines are the first-line diagnostic tests. If necessary, 24-hour urine analysis and imaging methods (CT, MRI, or functional imaging modalities (such as nuclear medicine methods)) are used to determine the tumor’s location and size.
Why Does Pheochromocytoma Occur?
The cause of pheochromocytoma is often unknown, but 30-40% are genetically based. These conditions are typically associated with genetic syndromes exhibiting autosomal dominant inheritance. MEN2, Von Hippel-Lindau disease, and SDH gene mutations are among the most common hereditary causes. In other cases, the tumor arises sporadically (spontaneously), and there may be no obvious risk factors.
What Are The Treatment Methods For Pheochromocytoma?
The primary treatment method for pheochromocytoma is surgical removal of the tumor. After the operation, symptoms such as high blood pressure, palpitations, and excessive sweating usually disappear.
However, before surgery, the patient’s hormonal balance must be medically prepared. Since the catecholamines secreted by the tumor can cause severe blood pressure fluctuations, these effects are first controlled with alpha-blocker medications. Subsequently, beta-blockers are used to regulate heart rate. This preparation process is critically important to prevent sudden blood pressure spikes (hypertensive crises) that might occur during surgery.
Uzm. Dr. Burcu Meryem Atak Sançmış evaluates each patient individually and creates the most appropriate treatment plan. After surgery, hormone levels are regularly monitored, and patients are placed under long-term observation for possible recurrences. In cases of hereditary pheochromocytomas, family screenings and genetic counseling form an important part of the process.
Frequently Asked Questions
What Should Be Considered For The Necessary Tests In The Diagnostic Process?
Patients should especially avoid alcohol, nicotine, and caffeine on the test day. Acute illness conditions can lead to false positive test results. The use of medications such as tricyclic antidepressants, dopamine-containing drugs (cabergoline), cocaine, selective serotonin reuptake inhibitors, selective norepinephrine reuptake inhibitors (SSRIs/SNRIs), alpha-adrenergic blockers, phenoxybenzamine, monoamine oxidase inhibitors, and sympathomimetic agents (amphetamines, albuterol) can cause falsely elevated metanephrine and catecholamine levels. Medications that could affect test results should be appropriately discontinued approximately 5-14 days prior, depending on the drug’s half-life.
Does The Disease Recur After Tumor Removal?
The risk of recurrence is higher in genetic forms. Therefore, regular follow-up and controls are important.
Can I Exercise After Being Diagnosed With A Pheochromocytoma?
Until diagnosis, strenuous exercise is not recommended because hormone secretion can increase with exertion. After treatment, controlled exercise can be performed as advised by the physician.
Do I Need To Take Hormones After Surgery?
If both adrenal glands have been removed, the patient needs lifelong cortisol and mineralocorticoid hormone supplementation.
Will My Blood Pressure Completely Normalize After Tumor Removal?
In most patients, yes, blood pressure normalizes. However, in some patients, hypertension may persist and require additional treatment.
Can Delivery Be Performed If A Pheochromocytoma Is Present During Pregnancy?
If the diagnosis is made during pregnancy, the safety of the mother and baby is the priority. Generally, a C-section is preferred. Medical treatment before delivery must be performed.
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