Adrenaline secreting tumour - Phaeochromocytomas and Paragangliomas

Paragangliomas form in nerve tissue in the adrenal glands and near certain blood vessels and nerves. Paragangliomas that form in the adrenal glands are called Phaeochromocytomas. Paragangliomas that form outside the adrenal glands are called Extra-adrenal Paragangliomas. In this information sheet, they will be called Paragangliomas.

Phaeochromocytomas and Paragangliomas may be benign (not cancer) or malignant (cancer).

Phaeochromocytoma

Phaeochromocytoma forms in the adrenal glands. There are two adrenal glands, one on top of each kidney in the back of the upper abdomen. Each adrenal gland has two parts. The outer layer is called the adrenal cortex and the inner or middle part is called the adrenal medulla.

Phaeochromocytoma is a rare tumour of the adrenal medulla and it usually affects one of the adrenal glands but it can affect both. Sometimes there is more than one tumour in the affected adrenal gland.

The adrenal medulla makes important hormones called Catecholamines. Adrenalin and noradrenaline are the two types of Catecholamines and they help control your heart rate, blood pressure, blood sugar and the way your body responds to stress. Sometimes, a Phaeochromocytoma will release extra adrenaline and noradrenaline into the blood stream which causes you to feel or experience certain signs and symptoms of disease.

Paragangliomas

Paragangliomas form in nerve tissue near certain blood vessels; these are rare tumours and can form near the carotid artery, along nerve pathways in the head and neck and on other parts of the body. Like Phaeochromocytomas, some Paragangliomas can make extra Catecholamines, those being adrenaline and noradrenaline. The release of these hormones into the blood stream may cause signs and symptoms of disease.

Signs and symptoms of Phaeochromocytoma and Paragangliomas

Some of these rare tumours that do not make extra adrenaline or noradrenaline will not cause any signs or symptoms and may be found incidentally when you are being examined for another reason. The signs and symptoms of Phaeochromocytoma and Paragangliomas occur when too much adrenaline or noradrenaline is released into the blood stream. These are the signs and symptoms you may experience. You may experience some or all of the signs and symptoms listed below:

• High blood pressure
• Headache
• Heavy sweating for no known reason
• A strong, fast or irregular heartbeat
• Being shaky
• Being extremely pale

Certain inherited disorders can increase the risk of Phaeochromocytoma or Paragangliomas and your doctor will discuss with you whether you will need genetic testing. The inherited disorders or gene changes listed below increase the risk of Phaeochromocytoma or Paragangliomas:

• Multiple endocrine Neoplasia 2 Syndrome, types A and B (MEN2A and MEN2B)
• Von Hippel-Lindau (VHL) Syndrome
• Neurofibromatosis Type 1 (NF1)
• Hereditary Paragangliomas Syndrome
• Carney-Stratakis Dyad (Paragangliomas and Gastrointestinal Stromal Tumour (GIST)
• Carney Triad (Paraganglioma, GIST and Pulmony Chondroma)

Diagnosis of Phaeochromocytoma and Paragangliomas

The following tests and procedures may be used:

• Physical examination and history: An examination of your body to check for general signs of health, including checking for signs of disease such as high blood pressure or anything else that seems unusual. A history of your health habits and past illnesses and treatments will also be taken.
• Plasma metanephrines: A blood test to measure adrenal levels in your blood. You may be asked to attend hospital for these tests and sometimes you will be asked to fast before these blood tests.
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside your body, such as the neck, chest, abdomen and pelvis. A computer linked to an x-ray machine will form the pictures. A dye may be injected into your vein or swallowed to help the organs or tissues show up more clearly.
• MRI (Magnetic Resonance Imaging): This machine uses a strong magnet, radio waves and a computer to make a series of detailed pictures of areas inside your body.
• MIBG scan or FDG PET CT: These are also specialised scans to form pictures of the inside of your body and the procedure will be fully explained to you by your doctor before you are sent for one. The MIBG scan is performed over two days and is referred to as a nuclear medicine scan. This is because you will be radioactive for a short period after the scan and this will require you to take contact precautions. All of these details will be outlines in an information sheet if you are referred for this test.

Once you have the diagnosis of Phaeochromocytoma or Paragangliomas made, your doctor will discuss your treatment. If you are experiencing some of the signs or symptoms mentioned previously such as high blood pressure, you may have to attend the hospital to start some medication for this and it will need to be monitored by the doctor looking after you. This will be fully explained to you before you start any treatment.

Your GP will be informed of all your investigations and any treatments that you start so please contact your GP or the Endocrine Clinic if you have any further questions. The endocrine nurses’ telephone number is 01223 217848 and please leave a message with your hospital number if we do not answer.