What Are Phaeochromocytomas?
Phaeochromoytomas (pheochromocytomas), often known as ‘phaeos’ (‘fee-ohs’), are rare tumours of the adrenal gland which develop in the inner section of the gland called the medulla.
The body has two walnut-sized adrenal glands, sitting on top of each of the kidneys (‘adrenal’ means next to the kidney). Even though the glands are small they are important as they produce several essential hormones. The medulla (the area of the adrenal gland from which phaeos arise) produces a number of hormones called catecholamines, mainly adrenaline (epinephrine) and noradrenaline (norepinephrine). These hormones help the body deal with maintaining blood pressure, deal with sudden stress or threats. Phaeochromocytomas may produce excessive amounts of these hormones, causing a variety of symptoms that can affect the whole body.
What is known about phaeochromocytomas?
- Phaeochromocytomas are rare, occurring in 1 per 100 000 people per year
- The majority (approximately 90% or 9 out of 10) are benign tumours (i.e. not cancer)
- The most common age for diagnosis is between the ages of 30-60 years of age, although 10% arise in children
- There is no sex difference or racial disposition
- Tumours occurring in children are more likely to be related to an inherited condition than those occurring in adults
Is there a genetic link?
- The majority of phaeochromocytomas are not linked to a genetic condition and are therefore known as ‘sporadic’
- Approximately 25% (1 in 4 cases) are caused by a gene change (mutation) that leads to an increased risk of developing phaeochromocytoma. The gene change can be passed down in families
- The inherited forms can occur in syndromes, including phaeo para syndromes (PPGL) [this site], multiple endocrine neoplasia 2 (MEN2), neurofibromatosis (NF) and von-hippel lindau syndrome (VHL).
What are the symptoms of these tumours?
Although they can grow for several years without serious symptoms, once functional, phaeos can cause a wide variety of symptoms which are mainly related to the release by the tumour of excessive amounts of hormone, mainly adrenaline and noradrenaline. Symptoms can occur intermittently often in episodes lasting only 15 minutes and may include some or all of the following:
- Facial paleness (pallor)
- Excessive sweating
- Racing heart rate (palpitations)
- Panic attacks/ sense of doom
- Weight loss
- Heat intolerance
- High (and rarely low) blood pressure (sustained or episodic)
- Nausea (with or without vomiting)
Many of the symptoms listed above are related to the high blood pressure (hypertension) that can result from this tumour. It is thought less than one case of hypertension in every 1000-2000 patients is due to phaeochromocytoma. Hypertension in patients with undiagnosed phaeos can be very difficult to control; however, once a diagnosis is made, special drugs are widely available to help control it.
For more detailed information on tests and treatments for phaeochromocytomas, please download the book below
Managing Through Hormone Surges
For the inaugural awareness week for phaeochromocytoma and paraganglioma syndromes, our Counsellor, Kym Winter, has developed an informational video and complementary short videos with different techniques to try to help yourself through hormones surges of functional tumours.
*These techniques are not intended to replace any medicine prescribed or advice given by your specialist.
Hormone Surges: Managing Anxiety and Riding Out the Wave
Progressive Muscle Relaxation
Four Square Breathing
Focus and Ground
5, 4, 3, 2, 1
In 2019 AMEND produced a new book on Phaeochromocytomas that can be downloaded for free below:
Author: Jo Grey, AMEND CEO, with the help of the AMEND Medical Advisory Team
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