Published: Sunday December 29, 2013 MYT 12:00:00 AM
Updated: Sunday December 29, 2013 MYT 10:29:59 AM

Speaking of lumps ...

Pheochromocytomas arise from the adrenal gland, and may secrete higher than usual amounts of hormones (epinephrine and norepinephrine), thereby causing havoc in the body.

Pheochromocytomas arise from the adrenal gland, and may secrete higher than usual amounts of hormones (epinephrine and norepinephrine), thereby causing havoc in the body.

When your blood pressure spikes, it may not be what you think it is.

WHEN I first started out on my “lumpy” journey, it was a lonely one as it was almost impossible to find a fellow patient. You see, I have a rare condition called paraganglioma.

Rare tumours that usually form in the peripheral nervous system, paragangliomas are often benign, though there are the even rarer exceptions.

The odd case or two may turn out to be bilateral carotid tumours, but I had – at the last count, and hopefully, it is truly the last – six tumours, in various parts of my body, growing along the parasympathetic chain.

In the last eight years, I underwent a series of surgeries (both invasive and non-invasive); the first epic one started the ball rolling, lasting 22 hours as there were complications.

In the process of excising my “lumpy additions”, I became the stuff of legends for medical journals, in Malaysia anyway.

I had published my experience in the hopes that it would create awareness of paragangliomas (My lumpy journey, Fit4life, Aug 12, 2012). And in the process, I stumbled upon others like me.

Thanks to the Internet, my story caught the attention of the “Pheopara Troopers”, a support group for those who suffer from this condition.

According to Dr Hew, pheos can come in various shapes and sizes, meaning there are different presentations and symptoms.

This US-based non-profit organisation was started by Amy Pitzer, Matthew Capogreco, Laura Becktel and Allen Wilson, whose personal experiences with the disease and collective interest led to the setting up of this patient-run outfit.

While it was heartening to share stories, it was also disconcerting to learn that there is another manifestation of paraganglioma, known as pheochromocytoma (or pheo for short), which comes from the same type of tissue.

With the former, the tumours tend to grow outside the adrenal gland, and apart from growing (very slowly) and obstructing passages, they are often hardly detectable.

Pheos, which grow in the adrenal gland, on the other hand, may secrete higher than usual amounts of hormones (epinephrine and norepinephrine), thereby causing havoc in the body.

Naturally, I consulted my vascular surgeon, who assured me that it was highly unlikely (although he did not dismiss the possibility altogether) for me to have pheos as I didn’t display any symptoms.

Apparently, if I did, the hormonal imbalance would have given off warning signals immediately.

But there could be a genetic link and my doctor suggested that perhaps at some point, it would be good that my children be tested, just in case it was in the genes.

But then, it struck me that Malaysia may not have facilities to test for this anyway. (Today, genetic testing for von Hippel-Lindau [VHL] disease and other hereditary links can be carried out at the Cancer Research Initiatives Foundation in Subang Jaya, Selangor.)

The Great Mimic

That lead me to a story that needs telling – what exactly is pheochromocytoma anyway?

Someone asked me why I wanted to pursue this “dry” subject, especially since the disease was so rare. But just as I found relief and comfort in better understanding my own condition, surely, pheo sufferers would be glad to finally have an answer for their unexplained symptoms.

The first time I had heard of it was on the TV series Grey’s Anatomy.

Scott Foley played a patient with VHL disease, and was diagnosed with a “dangerous tumour of the adrenal gland” called a pheochromocytoma.

Foley was great eye candy, but it was also an eye-opener as to how dangerous pheos can be, as they emit bursts of adrenaline at random intervals.

This can cause sudden spikes in heart rate or blood pressure, and can sometimes even lead to a heart attack or stroke.

The problem is, the symptoms often tend to masquerade as other diseases, and less experienced doctors may give the wrong diagnosis. Hence, pheo is sometimes referred to as “The Great Mimic”.

And no wonder, as ordinary symptoms such as headaches, sweating, and heart palpitations associated with hypertension, are usually primary indicators that something’s not right.

Consultant endocrinologist Dr Hew Fen Lee says that pheos “can come in various shapes and sizes”, meaning there are different presentations and symptoms.

“Many are classical symptoms, but even if they are, any doctor who doesn’t think of it will easily miss it. Thus, many patients often end up seeing a host of doctors before somebody stumbles on the right path and looks for it.

“The test for pheo may even turn out negative sometimes as the hormone may be secreted intermittently.

“Some patients only find out during an operation (for something else). There are also many who show no or mild symptoms.

“One patient was incidentally found to have a swelling in the adrenal gland and this subsequently turned out to be pheo,” he explains.

Drugs (tyrosine kinase inhibitors) may be prescribed to inhibit the formation of hormones by the tumour.

Alpha blockers to control blood pressure may be used as well.

In Malaysia, pheo cases are relatively rare, although not as rare as paragangliomas, which statistics indicate as one in 300,000 cases per year in the population. In the US, about 800 new pheo cases are diagnosed each year.

Pheos can also occur in combination with other tumours, conditions and in some familial (inherited) syndromes, and a series of tests can ascertain if specific hormone levels are not normal.

It is also associated with VHL syndrome, a rare, autosomal dominant genetic condition in which haemangioblastomas (blood vessel growths) are found in the cerebellum, spinal cord, and retina.

Usually benign

According to, pheos may occur at any age, but usually appear in those between their 30s and 50s, and approximately 10% occur in children.

Although the majority of cases are benign, about 10% of extra-adrenal pheos end up being malignant, especially when they occur in parts of the body that don’t contain chromaffin cells and are not part of the sympathetic nerve chain.

Those in the liver, lungs, or bones may be considered malignant with possible metastases to other organs.

“A malignant pheo is rare and very often, surgery is the only cure as other treatments don’t work so well.

The problem is whether they (pheos) are recognised and referred appropriately,” Dr Hew elaborates.

Scans such as MRI or CT scans will reveal any mass in the adrenal glands, and usually, surgery is recommended to remove the tumour(s).

In the case of malignant pheos, chemotherapy or radiation therapy may be required after surgery.

“Pheo is rare and the symptoms fairly non-specific. If you’re concerned, consult a doctor and request initial tests or referral to an endocrinologist,” he advises.

Unfortunately, as with any other paraganglioma, there’s no guarantee the tumour won’t recur after surgery, and the only way to manage pheo effectively is long-term consistent follow-up care.


Related story:

Dealing with the unseen

Tags / Keywords: Health, Pheochromocytoma, paraganglioma, blood pressure, hormones


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