​Pheochromocytoma

Pheochromocytoma is a tumour that develops in cells located in the centre of an adrenal gland (each of which is located on either kidney and produces hormones to control the functions of the body organs and tissues). It is rare and usually non-cancerous.

The presence of a tumour results in the excessive release of hormones that control the heart rate, metabolism and blood pressure, causing high blood pressure. The exact cause of this tumour is unknown. They usually develop in particular cells called the chromaffin cell, located in the centre of the adrenal glands.

Pheochromocytoma can occur at any age but they mostly occur from early to mid-adulthood. 

Pheochromocytoma can be life-threatening if untreated as they can cause massive damage to other body systems, especially the cardiovascular system.​

Common signs and symptoms of pheochromocytoma may include:

  • High blood pressure

  • Rapid heart beat

  • Excessive sweating

  • Severe headache

  • Chest pains

  • Paled face

  • Shortness of breath

Less common signs and symptoms may include:

The symptoms occur in brief spells, or attacks, when the tumour releases hormones. The attacks may last for 15 to 20 minutes and can occur several times a day.

Prompt diagnosis can lead to effective cure. Since the symptoms may be common for other diseases as well, request an appointment with an experienced doctor at the Internal Medicine Service Line at the Aga Khan University Hospital, if you have:

  • Persistent high blood pressure despite taking medications for controlling it

  • A family history of pheochromocytoma

  • A family history of genetic disorders that includes multiple endocrine neoplasia, type II (MEN II), von Hippel-Lindau disease, neurofibromatosis (NF1) or hereditary paraganglioma syndromes

Your time with your doctor maybe limited, so make sure to prepare for your visit beforehand. Here are some tips to help get you started.

Initially, your doctor may perform a physical exam followed by a series of test to determine the levels of adrenal, non-adrenal and by-products of these hormones.

Tests may include:

  • Urine test in which you may be asked to collect your urine for twenty-four hours to check the levels of catecholamines and metanephrines

  • Blood test to measure the levels of catecholamines and metanephrines

  • Computerized Tomography (CT) scan in which a series of detailed images, of the abdomen, are taken by a computer. A dye maybe injected in the vein or given orally to help the organs and tissues appear clearer.

  • Magnetic Resonance Imaging (MRI) of the abdomen in which a series of detailed images of the body are made on the computer using a magnet and radio waves

  • Positron Emission Tomography (PET) scan in which a small amount of radioactive glucose (sugar) is injected in the veins. Since the sugar is taken up by the tumour, the radioactivity will be concentrate there. This concentration will be detected by the PET scanner, which rotates around the body and an image will be produced. The tumour will appear brighter in the image

  • MIBG imaging in which a radioactive substance (tracer) is injected in the body, which is then taken up by the tumour. The scanner detects and confirms the presence of pheochromocytoma.

  • Genetic testing to determine if the cause of pheochromocytoma is an inherited disorder​

Your treatments options will mostly involve surgery to remove the tumour and a course of medications, if required, prior to the surgery.

Surgery involves laparoscopy which is a minimally invasive surgery. It includes a small incision to insert wand-like devices equipped with a camera and small tools which are used to remove the tumour and the entire adrenal gland, if necessary.

Medications may involve:

  • Alpha blockers to improve the blood flow and lower the blood pressure. This is done by preventing the non-adrenaline from stimulating the nerves and arteries so that they remain opened and relaxed

  • Beta blockers which reduces the work load on the heart and opens up the blood vessels. This lowers the heart rate and your heart beats with less force. This is done by inhibiting the effects of adrenaline

  • Other drugs that stabilizes the blood pressure if alpha and beta blockers fail to work ​

The Aga Khan University Hospital offers various support services to help with managing or recovering from the disease or condition. These include but are not limited to nutrition, physiotherapy, rehabilitation, specialized clinics and some patient support groups. Your doctor or nurse will advise you accordingly.
 
The Aga Khan University Hospital offers financial assistance to those who are in need and fulfil the eligibility criteria. For further information, you can contact the Patient Welfare Department. You can find the contact number of the Patient Welfare Department in the 'Important Numbers' section on the website homepage.
 
The financial counselling staff is available during office hours, at the main PBSD (Patient Business Services Department), to answer your financial queries on treatments' costs and authorize admissions on partial deposit as per hospital policies allow. The financial counsellor in the emergency room is open 24/7. You can find the contact number of the Patient Business Services in the 'Important Numbers' section on the website homepage.

Your doctor and or nurse will give you specific instructions about the prescribed medication. Please ensure that you take or use the prescribed medicine as advised. It can be dangerous to your health if you self-prescribe. Please inform the doctor or nurse beforehand if you have experienced any adverse reactions to any medications in the past. If you experience any symptoms of drug poisoning, overdose or severe reaction please contact the Pharmacy Service at The Aga Khan University Hospital immediately. You can find the contact number of the Pharmacy Services in the 'Important Numbers' section on the website homepage.




The information provided on our website is for educational purposes and not intended to be a substitute for medical advice, diagnosis or treatment. You should always seek the advice of your doctor or other healthcare professional provider.