Pheochromocytoma

A special kind of neuroendocrine growth known as “pheochromocytoma” develops from chromaffin cells. These cells are present in the adrenal glands and generate hormones that the body needs. Small organs, known as the adrenals, are situated above the kidneys in the upper portion of the belly. Pheochromocytomas form in the adrenal medulla, the innermost layer of the adrenals, in 80-85 percent of cases. Most cases of pheochromocytomas are benign, not cancerous. Only ten percent of pheochromocytomas move to various body regions. A pheochromocytoma typically only affects a single adrenal gland. The tumor produces hormones that might result in symptoms of a panic attack, including increased blood pressure, sweating, and a headache. Other bodily tissues may suffer severe or fatal impairment if a pheochromocytoma is not controlled. Blood pressure often returns to normal after a pheochromocytoma has been surgically removed.

Pheochromocytomas have unknown causative factors, according to researchers. In the middle of an adrenal gland are specialized cells known as chromaffin cells, where the tumor grows. Very few cases have a genetic history component, especially in young individuals. Genetic testing is helpful if pheochromocytoma is discovered younger than age 50.

Pheochromocytoma most frequently affects adults between the ages of 30 and 50. According to one estimate, just eight people out of every million people have pheochromocytoma. However, this number might be underestimated.

Pheochromocytoma risk is higher in those with some uncommon hereditary diseases. These conditions increase the likelihood that a tumor may be malignant. Both of the adrenal glands are also more prone to developing tumors. Some inherited conditions that increase the risk of developing pheochromocytoma are as follows:

Multiple Endocrine Neoplasias (MEN)

Multiple endocrine neoplasias, type 2, is a condition that causes tumors in many organs of the endocrine system. Pheochromocytomas are a component of both type 2A & type 2B of MEN.

Neurofibromatosis

This disease also results in pheochromocytoma. Numerous skin tumors, brown patches on the skin, and tumors of the eye nerve are all components of neurofibromatosis 1.

Von Hippel-Lindau Disease

This condition also increases your risk of having pheochromocytoma. The nervous system, endocrine, pancreas, and kidneys are only a few organs where Von Hippel-Lindau's disease may result in tumors.

Pheochromocytoma’s clinical features frequently include:

·         Elevated blood pressure

·         Headache

·         Heavy perspiration

·         A quick heartbeat

·         Shaky hands

·         Signs of a panic attack

·         Stress

·         Hazy vision

·         Pallor

·         Breathing difficulty

·         Constipation

·         Loss of weight

Blood tests

These tests check your body's levels of epinephrine, noradrenaline, or chemicals produced from those hormones, such as plasma metanephrine.

The 24-hour urine collection 

Every time you urinate for 24 hours, you must provide a urine sample for this test. A specialist will take samples for laboratory analysis.

Your doctor will probably request one or more diagnostic tests to look for a potential tumor if the findings of the laboratory testing point to the possibility of a pheochromocytoma. These tests might consist of:

MRI scanning

MRI creates precise pictures using radio signals and a magnetic field. Mri scanning is very useful when a diagnosis must be made for pregnant or pediatric patients. It can detect extra-adrenal mass as well.

CT scanning

It is an advanced kind of X-ray imaging. It is used to visualize the adrenal mass.

MIBG Scintigraphy 

It is useful when neither a CT nor an MRI scan can detect an adrenal mass.

Positron tomography

It is a scanner that may also be used to find radioactive substances that a tumor has absorbed.

Genetic testing

Genetic testing may be necessary to rule out a family condition if pheochromocytoma has been diagnosed.

Pheochromocytoma should be differentiated from other factors that can result in the same clinical features, such as:

·         Panic disorder

·         Factitious hypertension

·         Severe paroxysmal hypertension.

·         Carcinoid syndrome

·         A migraine headache

·         Overactive thyroid

• Renal hypertension

Doctors who evaluate and treat cancer (oncologists) and specialists in hormone problems (endocrinologists) may be needed to treat pheochromocytoma. Options for treatment to go through with your doctor include.

Medications

Your doctor could prescribe alpha-blockers and beta-blockers. These are medications to reduce high blood pressure to help you manage your symptoms.

Procedure

Surgery is required to get rid of the tumor as much as necessary. The whole adrenal gland may need to be removed in rare situations.

The adrenal gland can be taken out with open surgery. However, less aggressive methods include robotic surgery and laparoscopy. Once the blood pressure is kept under control, the stay in the hospital is between one and two days. After around two weeks, most people may resume their regular activities.

A typical laparotomy or open surgery is more complex. A hospital stay of five to seven days is necessary, followed by a four-week rehabilitation period.

Radiation and chemotherapy therapies are performed if pheochromocytoma has migrated to other bodily regions.

Rare yet dangerous catecholamine-producing tumors known as pheochromocytomas are nearly always fatal if not properly discovered and treated. The five-year survival percentage for patients with a tiny pheochromocytoma that has not migrated to other body areas is around 95%. 

·         There are certain foods that ought to be avoided if you have pheochromocytoma. Tyramine, a chemical that influences blood pressure, is present in foods and can exacerbate symptoms.

·         Your dietician will suggest how many servings of fat, oil, and sweets you must include in your diet.

·         Blood sugar adjustments may be needed due to an adrenal pheochromocytoma. You must work with a dietician to determine the meals that will help you regulate your blood sugar levels.

·         Products that have been fermented, pickled, preserved, cured, overcooked, or spoilt frequently contain tyramine. Some of these foods are cheeses and smoked meat.

·         To control your symptoms, you must avoid foods like coffee and tea that can raise catecholamine levels.

·         Other foods to avoid include bananas, chocolate, and fruit with citrus.

·         Calming breathing techniques may help with symptoms.

·         Meditation and yoga are essential.

·         You might lessen your tendency to think about unpleasant circumstances by discussing them with friends or family or by keeping a journal of your experiences.

Our clinical experts continually monitor the health and medical content posted on CURA4U, and we update our blogs and articles when new information becomes available. Last reviewed by Dr.Saad Zia on April 24, 2023.

Reference

https://www.nejm.org/doi/pdf/10.1056/NEJM198411153112007

https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-endocrine-tumor/pheochromocytoma#:~:text=Pheochromocytoma%20is%20a%20type%20of,on%20top%20of%20the%20kidneys.