Pheochromocytoma – Stages, symptoms, and causes

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What is Pheochromocytoma?

Pheochromocytoma is a rare type of tumour that develops in the adrenal glands. The adrenal glands are small organs that sit on top of the kidneys and produce hormones such as adrenaline.

Pheochromocytomas are usually benign (non-cancerous) but can sometimes be cancerous (malignant). They can produce excessive amounts of adrenaline, leading to symptoms such as high blood pressure, rapid heartbeat, anxiety and headaches.

Pheochromocytomas are more common in people with certain inherited conditions, such as multiple endocrine neoplasia type 2 (MEN 2) and von Hippel-Lindau disease. They are also more common in people with certain medical conditions, such as neurofibromatosis type 1 (NF1).

pheochromocytoma

Stages of Pheochromocytoma

Pheochromocytoma is classified into one of four stages based on the location and size of the tumour and whether it has spread to other parts of the body. The stages of Pheochromocytoma are listed below.

Localized Pheochromocytoma

Localized Pheochromocytoma is a type of Pheochromocytoma in which the tumour is contained within the adrenal gland. Localized Pheochromocytoma is typically classified as stage 1 or 2, depending on the tumour size.

Localized paraganglioma

Localized paraganglioma is a type of paraganglioma that is contained within the organ or tissue in which it originated and has not spread to other parts of the body. Paragangliomas are rare tumours that develop in cells called paraganglia, which are found in various locations throughout the body.

Regional Pheochromocytoma

Regional Pheochromocytoma is a type of Pheochromocytoma in which the tumour has spread outside of the adrenal gland to nearby lymph nodes or organs. Regional Pheochromocytoma is typically classified as stage 3 pheochromocytoma.

Metastatic Pheochromocytoma

Metastatic Pheochromocytoma is a type of Pheochromocytoma in which the tumour has spread to distant organs or tissues. Metastatic Pheochromocytoma is typically classified as stage 4.

The stage of the tumour is essential in determining the best course of treatment.

Symptoms of Pheochromocytoma

Symptoms of Pheochromocytoma are listed below.

1. High blood pressure

Pheochromocytoma can cause high blood pressure, which headaches, dizziness, and chest pain may accompany.

2. Rapid heartbeat

Pheochromocytoma can cause an irregular or rapid heartbeat, which may accompany chest pain or shortness of breath.

3. Headache

Pheochromocytoma can cause severe, persistent headaches.

4. Sweating 

Pheochromocytoma can cause excessive sweating, especially during physical activity or stress.

5. Anxiety 

Pheochromocytoma can cause anxiety or feelings of nervousness.

Causes of Pheochromocytoma

The cause of pheochromocytoma is not fully understood, but research suggests that it may be related to certain genetic changes or inherited genetic conditions.

In some cases, pheochromocytoma may also develop in people who have certain medical conditions, such as multiple endocrine neoplasia (a condition that affects the endocrine system) or von Hippel-Lindau disease (a rare inherited disorder).

It is important to note that most cases of pheochromocytoma occur spontaneously, without any known cause.

Diagnosis of Pheochromocytoma

Diagnosis of Pheochromocytoma may involve blood and urine tests to measure hormone levels, imaging tests such as CT and MRI scans to visualise the adrenal glands and a biopsy to confirm the diagnosis.

It is important to see a healthcare provider for a proper diagnosis and treatment of Pheochromocytoma.

24-hour urine test

A 24-hour urine test is a diagnostic test that measures the levels of certain substances in your urine over a 24-hour period.

A 24-hour urine test can be used to help diagnose Pheochromocytoma, a rare tumour that develops in the adrenal gland and produces excess amounts of certain hormones, including epinephrine and norepinephrine.

Blood catecholamine tests

Blood catecholamine tests are used to measure the levels of catecholamines in your blood.

These tests are often used to help diagnose Pheochromocytoma. The sample will be sent to a laboratory for analysis, and the results will be used to help diagnose or monitor Pheochromocytoma.

CT scan (computer tomography scan)

An imaging examination known as a CT scan (computed tomography scan) uses a combination of x-rays and a computer to produce precise images of the inside of the body.

MRI Scan

An MRI (magnetic resonance imaging) scan is a medical imaging test that uses a powerful magnet and radio waves to produce detailed images of the inside of the body.

To perform an MRI scan for Pheochromocytoma, you will lie on a table that slides into a narrow, tube-like machine. The machine will produce a strong magnetic field and radio waves, which will create detailed images of your body.

The MRI scan is usually painless and takes only a few minutes to complete.

Treatment of Pheochromocytoma

The primary treatment for Pheochromocytoma is a surgery to remove the tumour. This is usually the most effective way to control the symptoms of Pheochromocytoma and prevent complications.

Before surgery, your healthcare provider may recommend medications to help control your blood pressure and other symptoms. These medications may include alpha blockers, beta blockers and calcium channel blockers.

Genetic counselling

Genetic counselling is a process in which a healthcare provider or trained genetic counsellor discusses an individual’s risk of inherited conditions with them and their family.

Pheochromocytoma can be inherited in some cases, and genetic counselling can help individuals understand their risk of developing the condition and the options available for managing that risk.

During a genetic counselling session, the healthcare provider or genetic counsellor will ask about your family medical history and any genetic testing that has been done. They will also explain how Pheochromocytoma is inherited and what the risks are for you and your family.

Surgery

Surgery is the primary treatment for Pheochromocytoma. The surgery is usually performed under general anaesthesia, so you will be unconscious and unable to feel pain during the procedure.

Radiation therapy

Radiation therapy is a treatment that uses high-energy beams, such as x-rays or protons, to kill or shrink the growth of cancer cells.

Radiation therapy may be recommended for Pheochromocytoma in some instances, such as when surgery is impossible or the tumour has spread to other body parts. It may also be used to help relieve symptoms or control the growth of the tumour in some cases.

Radiation therapy

Chemotherapy

Chemotherapy is a treatment that uses drugs to kill cancer cells or stop them from dividing and growing.

Chemotherapy is usually given in cycles, consisting of a series of treatments followed by a period of rest.

Embolization therapy

Embolization therapy is a treatment that involves blocking the blood supply to a specific area of the body.

During embolization therapy, a healthcare provider will thread a small tube (catheter) through a blood vessel in your groin and guide it to the tumour in the adrenal gland.

Targeted therapy

A therapeutic technique known as targeted therapy employs drugs or other chemicals to target particular cancer cells while sparing healthy cells harm. Medical professionals use targeted therapies to treat metastatic and recurring Pheochromocytoma.

Prevention of Pheochromocytoma

There is no sure way to prevent Pheochromocytoma. However, you can do certain small things to reduce your risk of developing the condition.

Maintain a healthy lifestyle

Eating a healthy diet, streneously performing exercise and not smoking can help reduce your risk of developing Pheochromocytoma and other health conditions.

Monitor your blood pressure

High blood pressure is a common symptom of Pheochromocytoma. Monitoring your blood pressure regularly and controlling it with lifestyle changes and medications can help reduce your risk of developing the condition.

Know your family medical history

Pheochromocytoma can be inherited in some cases. Knowing the medical history of your family members can help you understand your risk of developing the condition and take steps to manage that risk.

Discuss genetic testing with your healthcare provider

Discussing genetic testing with your healthcare provider may be helpful if you have a family history of Pheochromocytoma or are concerned about your risk of inherited conditions. This testing can help determine your risk of developing Pheochromocytoma and other inherited disorders.

Seek medical attention for unusual symptoms

If you experience symptoms such as high blood pressure, rapid heartbeat, headache, sweating and anxiety, it is important to seek medical attention for quick recovery. These symptoms may be signs of Pheochromocytoma or other health conditions that need to be treated.

When to consult a doctor?

It is essential to consult a doctor if you experience any unusual symptoms that may be related to Pheochromocytoma.

They can perform a physical examination, order diagnostic tests, and determine the cause of your symptoms. Early diagnosis and treatment of Pheochromocytoma can help prevent complications and improve your chances of a successful outcome.

Conclusion

The good news is that Pheochromocytoma is typically harmless and curable. There is a strong connection between several genetic diseases and Pheochromocytoma, even if the majority of cases have an unknown origin.

It’s critical to undergo genetic testing if you or a first-degree relative has been diagnosed with a pheochromocytoma to rule out any inherited conditions that might, under certain circumstances, result in other health problems.

Speak with your healthcare practitioner if you have any inquiries concerning your risk of pheochromocytoma development.

FAQs

What triggers Pheochromocytoma?

Pheochromocytoma is a rare tumour that develops in the adrenal gland and produces excess amounts of certain hormones, including epinephrine and norepinephrine. It is triggered due to certain genetic and environmental factors.

What are the stages of Pheochromocytoma?

1. Localized pheochromocytoma
2. Regional pheochromocytoma
3. Metastatic pheochromocytoma
4. Recurrent pheochromocytoma


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