Pheochromocytoma

1 What Is Pheochromocytoma?

Pheochromocytoma refers to the rare tumors that develop in the inner medullary region of the adrenal glands. The adrenal glands are located near the kidneys. Although they are smaller in size, they have many functions. Cells in different regions of the adrenal glands carry out different tasks. The outer portion is referred to as the adrenal cortex. A part of this portion secretes cortisol, which is needed to handle stress. Another area secretes aldosterone, which regulates water and salt, and controls blood pressure. The inner area is referred to as the adrenal medulla. It secretes catecholamine, which is the “fight or fight” hormone. Over-production of these cells causes pheochromocytoma. Tumors from these catecholamine-secreting cells causes the clinical signs and symptoms of pheochromocytoma. If pheochromocytoma is caused by the chromaffin cells located outside the adrenal gland, they are referred to as extra-adrenal pheochromocytoma, or paragangliomas.

The adrenal medulla produces catecholamine hormones, adrenaline, and norepinephrine, all of which play an important role in rapid physiological changes during a stress response in the body. They increase the heart rate, flow of blood, and metabolism.

Pheochromocytoma results in excessive production of epinephrine and norepinephrine, leading to very high blood pressure. This may cause severe damage to the cardiovascular system.

Pheochromocytoma is most commonly seen in individuals aged 20-50 years. Treatment involves surgical removal of the tumor from the medullary region. Fortunately, pheochromocytoma is rare and most of its tumors are benign. 

2 Symptoms

Symptoms of pheochromocytoma are a result of the excessive production of catecholamine hormones.

The main symptoms of this condition include:

  • High blood pressure
  • Headache
  • Perspiration
  • Palpitations
  • Tremor
  • Facial pallor

The frequency and severity of hypertension may vary and are often difficult to manage with medications. Episodes of hypertension may result in cardiac arrhythmia, myocardial infarction, and even death. Some of the uncommon symptoms of the disease include:

Some people never develop symptoms. In almost 10% of cases, this condition was found accidentally or when the patient was undergoing diagnostic studies for other conditions. In some instances, the blood pressure is consistently high, whereas in others, the blood pressure comes and goes, thus making it difficult to record. Cases of high blood pressure were diagnosed with pheochromocytoma In only about 0.2% of people.

The sympathetic system mainly governs the release of “fight or flight” hormones. These hormones are released in response to stress or fear.

Excessive production of catecholamines may occur spontaneously or may be triggered by certain events like:

  • Physical exertion
  • Trauma
  • Anesthesia
  • Surgery
  • Foods with high levels of tyramine
  • Drugs, withdrawal from drugs, panic attacks, spinal cord injuries, and certain medications like decongestants, monoamine oxidase inhibitors, and stimulants

Pheochromocytoma may be a component of a hereditary condition called multiple endocrine neoplasia, or MEN. There are two types, MEN 2A and MEN 2B, and both are associated withpheochromocytoma. They are genetic syndromes that are transmitted from parent to child in an autosomal dominant manner. Both increase the risk of medullary carcinoma of the thyroid. Genetic testing can be done if there is a family history of MEN. MEN 2A not only carries the risk of pheochromocytoma, but also tumors of the parathyroid (glands near the thyroid that help regulate calcium levels). Other genetic disorders, such as von Hippel-Lindau syndrome and neurofibromatosis type 1, are also associated with the development of numerous benign and malignant tumors. 

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3 Causes

The definitive cause of pheochromocytoma is not known. Many of these tumors are sporadic in origin, while some are produced by inherited mutations.

About ten different genes have been linked to the development of pheochromocytoma. Certain syndromes like neurofibromatosis, hereditary paraganglioma syndrome, and Von Hippel-Lindau disease are also associated with adrenal tumors. 

4 Making a Diagnosis

Anyone with the classic triad of symptoms–headache, sweating, and heart palpitations—especially with high blood pressure may have a possibility of developing pheochromocytoma. If the patient knows his or her family history, he or she can inform the doctor of other types of endocrine tumors, including pheochromocytoma, if diagnosed in the family. If the doctor suspects familial pheochromocytoma, genetic testing may be performed. However, if suspicions are high, the physician may proceed directly with a series of tests to measure the hormones and their breakdown products.

Measures of catecholamine hormones, epinephrine, and norepinephrine in the blood and urine are indicative of pheochromocytoma. Catecholamine and metanephrine are measured in a 24-hour urine collection. Metanephrine can also be measured in the blood.

Once there is a possibility of adrenal tumors, imaging techniques are used to locate the tumor in the adrenal glands.

CT scans, X-rays, MRIs, and PET scans are commonly used to detect the presence of tumors. If detected, surgery may be needed. If no mass is seen through the imaging, or if it is unclear whether or not the mass is functional and clinically related to the findings, a test called 131-I-MIBG is used. In this test, a radioactive molecule is injected and localized in the tumor area, which makes it possible to visualize the pheochromocytoma on imaging studies.

In some rare cases, tumors may be detected while screening for other indications.

The hereditary nature of the disease is checked with the help of genetic tests. 

5 Treatment

Surgical removal of the tumor is the best way to treat the symptoms of pheochromocytoma.

Blood pressure and pulse are stabilized before surgery. This is the trickiest part of care. After anesthesia is given to the patient, there is a chance of an acute hypersensitive crisis developing. Therefore, blood pressure is managed with special medications before and during the surgery. Blood pressure is monitored throughout the procedure. A consultation with an endocrinologist is recommended to help design treatment for individual patients.

Medications recommended for lowering blood pressure include alpha and beta blockers. Alpha blockers are used before beta blockers. They help balance and control the blood pressure in response to anesthesia.

If the tumor is malignant, chemotherapy and radiation therapy may be needed.

Treatment outlook is very good, particularly if the tumor is not malignant and there are no complications after surgery.

Tumors recur in about 10% of the cases.

Clinical trials of specific or targeted new drugs called tyronisae kinase inhibitors have shown some promise in treatment. Drug therapy may not cure the patient, but it may reduce the symptoms and prolong life.

Prognosis

Generally, pheochromocytoma is benign and, if surgical complications related to blood pressure are avoided, the likelihood of being cured is excellent. There are chances of recurrence of malignant as well as benign pheochromocytomas after surgery; the recurrence rate is, on average, 10%. Therefore, follow-up care after surgery, along with additional appropriate treatments, is very important. If pheochromocytoma is diagnosed during pregnancy, the mortality of both the mother and fetus is high. Hence, the patient should be referred to a major center as soon as possible.

The outlook depends on whether pheochromocytoma is cancerous or not. People who had pheochromocytoma that wasn’t cancerous have a 96% five-year survival rate. To ensure successful treatment, seek out a surgeon who is highly skilled and can handle possible complications. 

6 Prevention

In many cases, pheochromocytoma is inherited, so it is not possible to prevent its occurrence. However, genetic testing can aid early diagnosis.

The actual cause of the disease is not known. Early treatment of the condition can prevent the spread of the disease to other regions.

7 Alternative and Homeopathic Remedies

Ayurvedic medicines based on herbs and minerals are used as an alternative remedy for pheochromocytoma.

Thuja and Natrum mur are suggested in homeopathy for alleviating symptoms of the adrenal tumor. 

8 Lifestyle and Coping

Early diagnosis and treatment are very important in order to control the symptoms of pheochromocytoma and cope with the condition.

  • Diet: Eat a variety of healthy foods. This will help you feel better and give you more energy.
  • Check blood sugar: Check your blood sugar using a glucose monitor several times a day.
  • Drink liquids: Drink adequate amounts of liquid each day, including water, juices, and milk.
  • Manage stress: Meditate or listen to music. This will help you cope with your stress.
  • Rest: Do not perform your regular activities too quickly. Rest is very important in order to recover. First, take up your activities again slowly, and as you feel stronger, do more. Sleep at least six to eight hours each night.
  • Take medicines as directed: If your medicine is not working, call your primary healthcare provider. Disclose any allergies and make a list of medicines, herbs and vitamins for the doctor. Being compliant with medications and checkups helps to improve the success rate of the treatment.

Recurrence is seen in 10% of cases, thus, follow-up after treatment is essential to prevent complications. 

9 Risks and Complications

There are several complications associated with pheopchromocytoma.

High blood pressure due to pheopchromocytoma may lead to:

Treating pheochromocytoma with surgery may cause complications. Some conditions, such as high blood pressure crisis, low blood pressure crisis, and irregular heartbeat, may develop. Surgery affects the major hormones in the body.

Contact your caregiver if:

  • You have chills or feel weak and achy
  • You experience nausea or vomiting
  • You are unable to make your radiation or chemotherapy appointment
  • Pain is worse or pain medications are not working

Skin is itchy, swollen, or has a rash

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