Pheochromocytoma is a tumor from the inner part of the adrenal gland. Each person has two adrenal glands, one on each side of the body. The adrenal gland is located on top of the kidney (thus the name ad-renal). The adrenal gland has an outer layer and an inner core. The outer layer is called cortex while the inner core called medulla. Pheochromocytoma is a tumor from the adrenal medulla. There are other cells in the body that are very similar to the cells in adrenal medulla and they can also produce pheochromocytoma as well. In that case, the pheochromocytoma is called paraganglioma.
Pheochromocytoma makes substances called catecholamines. These substances constrict blood vessels, increase blood pressure, deplete the body of fluids, and hurt the heart. Catecholamines are also normally produced by the adrenal medulla. Only in excess amounts, catecholamines cause harm to the body.
Pheochromocytoma is indeed very rare. Because a common symptom of pheochromocytoma is hypertension, pheochromocytoma is often considered in patients with unusual hypertension. Another reason that pheochromocytoma is often an issue is adrenal mass identified through the wide use of abdominal imaging.
Pheochromocytoma is a tricky disease. It can be deadly if not diagnosed or treated properly. On the other hand, if diagnosed and treated properly, patients with pheochromocytoma can be completely cured. Although the most common presentation of pheochromocytoma is hypertension, pheochromocytoma can mimics many other diseases such as anxiety, palpitation, or heart attack. In a recent study, it is shown that more than 20% of patients who undergo surgical removal of a presumable pheochromocytoma end up not having pheochromocytoma. Similarly more than 20% of patients who indeed have pheochromocytoma are diagnosed as a dangerous surprise.
I welcome patients and colleagues to post comments or questions to this blog and we can go over all aspects of pheochromocytoma on a case-by-case basis. Please keep any personal information confidential. For example, even if you yourselves may have hypertension and a concern for pheochromocytoma, just say "a friend of mine (or my cousin Johnny), 45-year-old man, has hypertension for a year and his doctor tells him that he may have pheochromocytoma."
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If a person has had two pheos removed and has the SDHB gene, how often should the bichemical tests be run? I have heard every four months and I have heard once a year. Thank you
ReplyDeleteI will do tests every year or whenever you have symptoms. In addition, imaging should be done every few years as well.
ReplyDeleteDr. Pheo
This comment has been removed by the author.
ReplyDeleteHow can we be sure a person is suffering from Pheo? And how dangerous is the surgery?
ReplyDeletePlease read other posts. The answers are there.
ReplyDeleteHello,
ReplyDeleteAs you are obviously someone who knows alot/has seen a fair few pheos, have you ever known them to secrete other hormones, for example to be causing cushing's syndrome at the same time? I'd be interested in knowing the prevalence and mechanism of this, as the tumour would therefore need to be of mixed cell type wouldn't it?
Also, in your experience how common is it for pheochromocytoma patients to just have elevated metabolites of catecholamines? I have read that some pheos co-secrete the catechol-o-methyl transferase responsible for the metabolites - how common is this? Could this be the soul reason for having only elevated metabolites?
Many thanks.
Dear Annie,
ReplyDeleteSorry for the late response. Yes, pheo can secrete the hormone ACTH or cortisol that causes Cushing's syndrome. That phenomenon is rare, though.
The metabolites are always more sensitive than catecholamines. The reason is that some catecholamines do not make it to the blood but degraded inside the pheo cells.
Dr. Pheo
Hey doc....all started about 3 months ago...had sudden extremely rapid heart rate while at the movies...very pale...took to er and did standard heart workup blood draws ekg etc....doc said i was fine. A few weeks after I started having random attacks where I would be calm...not worried or thinking about anything....all the sudden I could feel pounding heart.. then rapid heart rate. Bp would shoot through the roof and would get very bad adrenaline shakes after it was over....I'm talking full body like I was being electrocuted shakes....this happened on and off for a few weeks...saw GP. Did blood. Gave me xanax. Still happening...but not as frequent. ...i was going through some trying times emotionally with family issues etc. Then one night last week I had 8 in a row...same signs and symptoms...break...more intense...shorter break...more intense...until I hd to again go to the er...where they just took bp and heart rate 150 over 90 and hr 122 ( my normal resting is 110 over 55 and hr around 55-low 60s). The did nothing...not even hook me up to ekg ma chine until an hour after I was there....gave me an ativan and sent me on my way....i sweat ( hands feet underarms anytime I am not at my house....i know this sounds like social or gad) in the past two weeks I have gotten maybe a few ml nights of sleep an hour...talked to a doctor I know at work one morning after not sleeping for a week and still wide awake at 6 am. Took a xanax he prescribed and woke up an hour later. Have lost 14 pounds in about 2 months. Don't have an increase in appetite. ..may be due to benzo I have been on and off of daily for the past month and a half. Have had thyroid checked and was told it is ok. Went to and endocrinologist and did chromogranin testosterone cortisol and adrenaline bloodwork....waiting on labs to get back. I've been written of as another anxiety patient by 3 doctors now....i did have anxiety attacks 12 yeas ago after being in a car accident and it's was nothing compared to what these attacks are like....i literally feel like they are going to kill me instead of ( fearing something is wrong ) like an anxiety attack. Symptoms are 10 times as harsh.....thoughts?? Thanks for your time -jared
ReplyDeleteDear Jared,
DeleteSorry for the late response. I have been away from the blog due to the holidays. Pheo is a real concern. Let's see the test results.
Dr. Pheo
Dr. Pheo.. a friend of mine has always been active, has been sick and unable to work for several months... The most prominent feature of whatever is wrong, he has been to er's and multiple doctors, are attacks that start each day around 11 a.m and subside around 2 pm or so and arise again about four oclock in the afternoon and end as darkness approaches, he often has spells where he is very light and temperature sensative.... he has had no formed stools for months ... he describes the attacks feeling like a panic attack that is so severe that he gets terrible right abdominal pain and sometimes a feeling of tightness in the center of his chest... obviously if he is stressed, this gets worse, also thinks his blood sugar is very unstable and also his electrolytes can easily become unbalanced... if he is more settled, he can exercise by either walking or riding a bike and the "attacks" are seemingly attenuated by the exercise and not so noticeable... several abdominal ct's have been done with no masses seen... but you can set your watch with the timing... Your thoughts?
ReplyDeleteDear Unknown,
ReplyDeleteI don't know but the likelihood of an adrenal pheo or other abdominal neuroendocrine tumors is low. I would still check plasma metanephrine to rule out pheo in the chest or bladder.
Dr Pheo
I was diagnosed with hypertension at the age of 24. It has now been 2 years of which the doctors have decided the hypertension is due to it running in my family. Throughout the 2 years I have had "spells" of dizziness, rapid heart rate, extremely high and fluctuating blood pressure, tremors, headaches, and anxiety. Recently I have been suffering from lower back, flank, and abdomal pain. Also recetly, my blood pressure medication has been increased. I have had tests to rule out thyroid problems, I have had blood tests and ultra sounds of my kidenys, I have had a 24 hour heart monitor test, as well as a abdominal ct, all which have come back normal (other than an ovarian cyst). Recently I have also been suffering from frequent kidney infections.
ReplyDeleteDoes the abdominal ct and the ultra sound of my kidneys rule out pheo?
Or should I have a 24 hour urinalysis done?
Dear Hannah,
DeleteIt is still prudent to do biochemical test to rule out pheo. Pheo outside the abdomen is rare. Heart and bladder are two of the places where pheo can be found.
Dr. Pheo
Dr. Pheo,
ReplyDeleteFor the last year I have been experiencing extreme anxiety for no apparent reason. It started off with random dizziness while standing but has escalated into severe attacks exacerbated by mundane situations like standing in line at the store. I feel like I am stuck in overdrive and do not feel at all myself. My tsh levels had been dropping over the last 6 months from 1.34 to .52. I also get intense adrenaline rushes along with blurred vision at the same time every night 9 pm, these episodes can last for a few hours to throughout the next day. I also have palpitations, lightheadedness, near syncope and failed at tilt table test at 13 minutes in what they called a neurogenic vasovagal suncope. A recent Ct showed a 2.9cm "myelolipoma" on my right adrenal gland. Do you think this could be a pheo? Any information would be greatly appreciated. Thank you in advance.
Dear ZigZag,
DeleteWhen a CT scan suggests myelolipoma, the adrenal nodule is very unlikely pheo. There are exceptions of course. You may want to test plasma metanephrines to be sure.
Dr. Pheo
I am currently waiting for the results of a plasma and 24 hour urine metanephrines test. Thank you for your reply. You website and blog are very informative. Keep up the good work!
DeleteZZ
Dear Dr Pheo
ReplyDelete3 months ago I went to ER after experiencing high BP 220/120 shivering, feeling very cold, high heart rate, almost passing out, weakness, light sensitivity. Episodes pass after 1 to 3 hours.
Many blood tests done, all normal. Going for a second 24h urine test. Abdominal ultrasound was preformed.
Since then, several smaller episodes.
Mostly characterized by suddenly feeling very cold and tired. Body temp 36. Not always always accompanied by high BP. Any advice appreciated.
Dear jaredjar,
DeletePheo needs to be ruled out. Let's see the 24 hour urine results.
Dr. Pheo
How can I get a doctor to order a lab test for plasma metanephrines? Are there any online docs that will do it?
ReplyDeleteDear Life with AJ,
DeleteAny of your doctors can order it if the doctor feels you may have pheo.
Dr. Pheo