Before I begin to share my epic battle with pheochromocytoma.. you have to know what this crazy disease is! Keep reading to understand what it is to be a ‘zebra’
WARNING – MEDICAL JARGON OVERLOAD 🤓
Pheochromocytoma is an extremely rare tumor that develops in cells in the Center of the adrenal gland (adrenal medulla). We all have two adrenal glands, one above each kidney. These two adrenal glands produce hormones that give instructions to virtually EVERY organ and tissue in the body. The adrenal medulla controls hormones that initiate the flight or fight response.
These two small glands impact every organ and tissue in your body. In other words, no adrenal glands, you die.
(Kind of a big deal)
But here’s the thing – the pheochromocytoma tumor actually releases these same hormones in lethal amounts, causing persistent or episodic high blood pressure and rapid heart rate. If left untreated, or (often undiagnosed!) it can lead to life threatening damage to other parts of the body (ex) stroke, heart disease, nerve damage in the eye, kidney failure, and even death.
Translation: The pheochromocytoma tumor makes you overdose without warning on adrenaline hormones!
Sometimes can be triggered by things such as movement, exercise, certain foods and drinks containing tyramine – breathing… and sometimes it’s just sporadic and can happen entirely unprovoked.
When the tumor releases its high amount of adrenaline into the body, it causes a severe and sudden crisis and this results in a spike of blood pressure and heart rate. The symptoms this causes may include:
- Severe onset headache
- heart palpitations
- severe pain in the body/eyes/head/joints
- chest pain resembling heart attack
- symptoms can vary depending on the individual – less or more
Some people may also find they can go days without an attack, months even, some may find their tumors are highly episodic and have attacks several times a day. Also, not everyone will have every symptom listed above, some people may just have a high heart rate and sweating for example, or high blood pressure and headache, it varies depending on the person. Some may not have any symptoms at all and get diagnosed by accident (this is rare).
Research still has not shown what exactly causes the development of this tumor. One thing they can confirm is the tumor is made up of cells called chromaffin cells, these produce and release certain hormones, primarily adrenaline (epinephrine) and noradrenaline (norepinephrine)
Adrenaline and noradrenaline are hormones that we actually need to function as the trigger for your body’s ‘fight or flight’ response to a perceived threat. (cool, right?) These hormones prompt higher blood pressure, a faster heart rate and a boost in other body systems that allow you to react quickly with a burst of energy. *Think, lion attack*!
The pheochromocytoma ultimately makes you overdose on these hormones, without warning, which we in the community ‘attacks’ or episodes. Essentially your tumor is attacking you with your own damn adrenaline, assholes. (the pheo, not you!)
Unfortunately these tumors can also invade the heart, head, neck, bladder, abdomen, and the spine. These little suckers can also produce dangerous hormones. However these tumors are then called paragangliomas, (a pheo that is not located on the adrenal gland)
There are several rare genetic mutations pheo patients are tested for following their illness, most people will find they have a familial genetic disorder which causes the pheos and also are more at risk ordinarily for it to reoccure.
(I however was not one of them, I tested negative for any genetic mutation. Making my pheo sporadic, which sometimes can be seen as a good thing, however some see it as another hindrance being unable to detect or explain why this happened to me)
Even more rarely.. a pheochromocytoma is cancerous (malignant), and the cancerous cells spread to other parts of the body (metastasize)
You will see under ‘My Very First Pheo‘ a little more in detail of how you get to this diagnosis of such a rare disease, but basically…
If you are having episodes of medication resistant high blood pressure, rapid heart rate, severe onset headache, shaking, sweating, flushing, going pale, vomiting, etc… and then you’re back to fabulous, your doctor SHOULD eventually take a hint that it may be time to do some testing for this disease.
Instead, most people are often misdiagnosed with depression, anxiety, insomnia, you catch my drift?
There are a few biochemical tests they need to run to test you for this tumour, one being a 24hr urine analysis (urine catecholamines) along with a special blood sample (plasma metanephrines). Chromogranin A should always be checked as well as dopamine (3MT)
there is such a thing as false negatives.. Chances are if the tumor is very active, it’ll show results through the roof – but ….. It can also be tricky and completely show nothing even though you are are highly symptomatic.
(yes, this happened to me, it actually happens to a lot of us) in which case if you’re sure of your symptoms (listed above) you need to be persistent and don’t give up with your doctor! You KNOW your body! It may be time to get imaging done and look for the tumor rather than just rely on the urine/blood tests.
Surgery is the preferred method of treatment for this tumor, even though it is an extremely risky surgery, this is all they can do to hopefully remove the tumor and have you back to some what normal.
Other treatments such as chemo and radiation are not used as a first line treatment simply because the tumor cannot be fully eliminated in it’s entireity. These treatment options are only used when the tumor is inoperable, to hopefully shrink the tumor and extend life. (These options are used when the disease is cancerous, metastatic, and surgery is not an option anymore)
Pheochromocytomas and paragangliomas are no longer classified as ‘benign’, as of 2017 WHO categorized them as non metastatic or metastatic. Which means they are a form of neuroendocrine cancer
All pheo paras require lifelong follow up, no exceptions! Even if sporadic and no genetic mutation is found, it can still recur.
Stay tuned for more info under my pages…
All my symptoms, just found the tumor May 25th 2017
What were your symptoms and how did they go about finding the tumor?
Thank you for your post! I have Been battling all of the symptoms off and on for years. Things got really bad though after having my daughter at 40. My Bp is resistant to meds (the current cocktail works) and my anxiety is now controlled with Zoloft though I feel It masks all of the other symptoms. One test a month ago showed elevated epinephrine after my Bp rose to 234/127 completely unprovoked. then the nephrologist tested me a month later and my labs (blood and urine) were normal. scans showed possible stenosis of my renal artery so they sent me for an angiogram only to find my arteries were fine, no narrowing at all. all of my doctors feel confused bc my symptoms for a Pheo are there but my tests are “normal” yet my Bp is high, I’m constantly flushed and sweating and I frequently Get headaches. I’m at a loss bc I know what I’m feeling is not “normal”. Thank goodness I have doctors that are hunting but now we wait for another episode to happen so insurance will pay for a pet scan.
Meredith, I’m so glad you found this blog and are currently receiving help for a diagnosis. Typically a good doctor will only rule out a pheo with someone that is symptomatic after testing three separate times, make sure they test you for plasma metanephrines and also chromogranin A. Chromogranin a is not specific to pheo but it indicates that there is SOME type of neuroendocrine tumor present. The tricky part is just finding it. This is the most important part, and a pet scan doesn’t always show it. Unfortunately with these scans unless your tumor is secreting that specific uptake for that specific scan, it won’t show. The BEST possible scans to find a pheo specific tumor is a gallium 68. They are the gold standard for this disease, they will find even the smallest tumors. Please push to have a gallium 68 and perhaps it will find what you’re looking for.
These symptoms are exactly what I was experiencing. I had 18 months of symptoms. A heart attack and a stay in CCU prompted testing. I’m now due surgery in 2 weeks for my adrenal gland removal to get rid of the 3.6cm tumour.
I’ve only just found this blog and am enjoying reading it, although so sad too.
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Hi, Did you have surgery to get ur tumor removed? I have a 5cm tumor on my right adrenal gland and they tried to perform surgery to remove it last week, but my blood pressure went up to 190/112 while they were putting my a line in 😔
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Yes they removed it and my whole adrenal gland. I’m well now :).
How about you? Are you scheduled for surgery?
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Hi, curious if what you are saying is that your blood and urine test did not show positive but a CT or MRI did for diagnosis? I did the blood test and it showed twice the normal limits, so they said because it didn’t show three times the normal range that it could not be Pheo? My question is, why would it be twice the normal limits? I’ll never understand Dr’s way of thinking. He said stress. I’m far from stressed. I’m super skinny from losing so much weight and have a ton of these symptoms. My DHEAS is over double the normal values. They say stress. Did you lose weight? I’ve lost a ton and have horrible anxiety…never did a year ago! I can say through all my testing I have found out that I do not detox well and I’m toxic to mold and plastics so I think toxicity plays a role. BTW, your blog is wonderful!
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I had the urine test done in the hospital when I was having sinus tachycardia but it was negative. Could being on tons of iv fluids, an anxiety medication the day before, and a beta blocker towards the end of the test show a false negative? Great blog, thanks and God bless.
I was having heat intolerance, random rapid heart beats, tremors, lost 30 pounds in 3 months (I’m 22 and now weigh 101), insomnia causing fatigue during the day. I thought I had hyperthyroidism since I work at an OBGYN clinic and see patients with that all the time. I was tested and it came back normal. Two days later, my NP (who had never heard of this herself) stated that my Doctor suggested this and I’m doing my 24 hour urine test on Sunday and bringing it to the lab on Monday. I had high blood pressure (140/70) and my WBC and Lymphocytes were crazy compared to normal. I’ve been doing so much research on this but I keep thinking, with something so rare, could I really have this?
I’ve been having the sporadic episodes. One was hours after my CT scan that found the tumor. One last year and now three in the last few months.Blood test was negative but my doctor says he still cannot rule a pheo out. On heart meds and seeing a cardiologist in a few weeks. Have not done the urine test. The episode after my CT scan had my BP and heart rate up for 10 hrs!! I thought it would be a “nope bloodwork was negative so no pheo. I’m just at a loss now as it still could be a pheo but more tests😞
I’m 39 and had my Pheo removed from my left kidney when I was 34,I guess I had symptoms most my life,from a young age I had a sensitivity to heat and suffered from headaches,in my teens it got that bad I had more days with a headache than without,it wasn’t until I was waking up screaming like I was being murderded that I got taken seriously,many nights in a hospital on morphine and tests,ct scans and lumbar punctures showed nothing but elevated blood pressure which they put down to the stress of pain,I was diagnosed with cluster migraines,in my late twenties out of the blue after a weird episode I am diagnosed with severe anxiety disorder which lasts for months am put on medication and after a while symptoms disappear as quick as they came….fast forward till I’m 34,get an itch under my skin that starts at my ankle and ends at the knee mainly bad at night don’t think much of it…after a few months I start getting heart palpitations still don’t think much of it…it’s a brutal summer so I have had this on occasion before(at first)palpitations get worse ,pass out at work,feel unwell…go to doctor not thinking any symptoms are related tell him about my racing heart he checks blood pressure,says it a bit high sees I “suffer from anxiety”could be under stress and prescribes me blood pressure tablets and to come back in a month…within days I’m worse,heart feels like it’s beating out of my chest,I last a little over a week before I’m back at his office,he’s on holiday( thank god)I see another doctor…she can’t understand why I’m on these at 34…were there other tests done?takes my blood pressure 3 times and freaks out 220/122 I’m a walking stroke,she orders priority tests and two days later I’m sitting in front of her and she’s telling me I have a 4cm tumour…what followed was 4 mths of testing at the hospital,genetic cancer testing more ct scans and a flight to a bigger hospital so I could have radiation dye injected in me to check my whole body for more tumours,was placed on 6 different medications to try and lower blood pressure…some meds made the tumour produce more adrenaline…blood test showed I had 1000 times more adrenaline in my body than normal ( not to mention all the other hormones were out)finally go in for op,blood pressure is down to160/100 not as low as the want cause it’s a risky op,tumour is removed fine,but when they put me under they accidentally nicked the artery in my neck and bled into my lung(this is another story)long story short I recovered well ( eventually)I no longer suffer from these headaches,the supposed anxiety is gone as well…but the blood pressure?it went down and stabilised but I will be on medication for the rest of my life for it,it went so long untreated I’m stuck with it 145/90 usually so not too bad…wish all u guys suffering the best of luck in your treatment
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Hi, I have been mis diagnosed with anxiety since I was 12 I am now 18. I recently got diagnosed with POTS syndrome. I am not getting any better and I’m on medication and following what my doctors tell me
To do. I have gotten so much blood work done and everything is coming back normal. After reading all about pheochromocytoma I am worried I have it since I am not getting any better and it’s been almost 8 months of being bed ridden. Normally to be diagnosed with pots syndrome they make you do a tilt table test, I was not tested that way a doctor just told me I had
Pots syndrome mimics Pheochromocytoma, and pheochromocytoma mimics POTS.
Do you have any recommendations on what I should do?
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To add I have every symptom of pheochromocytoma. I will be fine for a few days and be so sick I can’t move for a few days. Or I will be fine one minute and be sick the next. I am so frustrated
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Hi Nikki, I’m so sorry that you’re going through the extensive phase of unknowing. I know this is going to seem like an annoying answer: but if you’re not being heard by your current doctor, please find another one that will. I know that’s very difficult to find a different provider, but unfortunately if you’re not able to get the results you need from the current one, you most likely never will. And your health is too important to wait. In the meantime, I would ask for the tilt test you mentioned, and continue to push for labs such as… plasma metanephrines, 24hr urine catecholamines, and chromogranin A (blood) these will be the beginning indicators of what your biochemical values look like for the potential of adrenaline secreting tumors. It will not technically 💯 rule it out, if you’re symptomatic, but it will give you A baseline of where you stand. Have you had any imaging done? I would also urge you to join the support groups on facebook and share your symptoms and story, ask for advise from others who are in your area as far as a well informed specialist. You can also go to the Pheo para alliance and look under alternative diagnoses and get a comprehensive look of things that can mimic a Pheo or can be other options for diagnosis. I worry that if I you’re not responding to the POTS treatment it may not be all there is. I’m not super familiar with POTS other than hearing about it from other peoples experiences, so I don’t want to jump to any conclusions. There could be further tweaking that needs to be done to your treatment but as far as the Pheo is concerned, that’s where I would start. The testing I mentioned above. I hope this helps 🤍🙏🏼
Thankful to have found you! Finally found a dr that saw a spell in person and my blood pressure jump from 127/72 to 270/156 in a matter of 2 min! Tell her it was happening and giving a UA and bam it all hit. I was Referred to a hematologist/oncologist and he asked how my days are and I broke Down telling him and he said I’m positive it’s Pheocromocytoma and I now Have a biopsy 4-1-22. REWIND 2yrs ago I passed Out at the hospital while hubby was there with bp stroke range and that dr told us to tell my pcp about pheocromocytoma and she dismissed it as chronic bp and upped my already 3 bp meds to 4 and nitro. Noting I have Wrecked 2 vehicles due to blacking out from bp to high. I didn’t drive for almost 2 yrs. cleared to, and now it’s all started again 6 months ago. (This is happened for the last 7 yrs off and on now it’s daily.) Finger tips blue and freezing while the rest of my body profusely sweating.
Hi misti, I’m not sure if I read that right or if it was in the past but if they are trying to biopsy a known pheochromocytoma you need to STOP that procedure because it can be fatal. You don’t poke a grenade. If they know it’s a pheo and they’re recommending a biopsy, these doctors are not specialized in this disease because it’s known absolutely known that you cannot biopsy a secreting tumor. Just applying pressure on the tumor area from the outside of the body can make it secrete and put you in a catecholamine crisis. Please bring this to their attention, and get referred to a specialized ENDOCRINOLOGIST for pheo/para.
There’s so much information in the support group on Facebook, I’d recommend starting there and sharing your story as you did here to get support and even recommendations based on your area for a medical team.
You would need to begin to be blocked by an alpha and then a beta to control the episodes and block the output of catecholamines the tumor is secreting while doing the testing to confirm – they will begin with 24hr urine tests if they haven’t already and plasma metanephrines. and then they will proceed with nuclear IMAGING, not a biopsy!
Once they have collected all the pieces of your puzzle, they will proceed with surgery based on your results.
And then lifelong follow up, I’m glad you found me when you did.
Before seeing this dr I was Told it was multiple myloma and he said this is to fully exclude that. And that it will confirm the pheo. I had A hysterectomy 12weeks ago and the last 2 weeks my bp is somewhat normal at least for me. 180s/118 my heart rate runs low 35-50 normally because I smoke Weed instead of pain pills for the chronic pain (hardware in back, and I have Auto immune Celiac disease and that messes with my body).. I’ll go to the fb page and also I already Contacted my dr about not doing the biopsy. I have Had a abdominal ct scan with contrast and it showed nothing that was last week.
So I had A 24 hr urinalysis done and it shows my NORMETANEPHRINE and METANEPHRINES were high. Does it always mean you have pheochromeocytoma?? I was being evaluated for cushings and now I’m very worried about this.
Hi Anna, the chances are very high… but it depends on how elevated they are. If they’re 4x it’s a clinical diagnosis of pheo or para and they will move onto imaging to find the tumor