Pheo is a rare disease. In my practice, I see many patients who are suspected by their doctors of having pheo. After evaluating the patients, I generally find that only about 10% of them really have pheo, but the other 90% do not. The patients' response is usually predictable. Those who are diagnosed by me to have pheo are happy (and apprehensive, of course) after I discuss the nature of the disease and treatment plan. Those who do not have pheo are disappointed and frustrated. From the patients' point of view, they don’t care too much whether they have pheo or not per se, but they do want to feel better. If it is not pheo, what else is wrong?
About half of the patients for whom pheo is ruled out, especially young patients with hypertension, just have “essential hypertension”. Essential hypertension is a vague diagnosis simply meaning that the doctors don’t know why the patients have hypertension. Most patients with essential hypertension have a family history of hypertension. Their hypertension has multiple causes but none of the causes stands out obviously.
About a quarter of patients have obstructive sleep apnea (OSA). Patients with OSA are in a chronic stress mode so that they have catecholamine surges. OSA is pretty common and affects 5-10% of all people. Many people do not know they have OSA. The doctor has to probe whether the patient snores or has daytime somnolence. If the patient has suggestive symptoms, a consultation from a sleep specialist or pulmonologist is very valuable. The effects of CPAP treatment can be dramatic.
Another quarter of patients have anxiety disorder. People tend to have negative emotional reactions toward anxiety disorder. Some would wonder why they have the disease if they are not really anxious about anything. Anxiety disorder is a disease that we don’t know the cause of, just as depression is a disease that we don’t know the cause of. Patients should be evaluated by a psychiatrist to make the anxiety disorder diagnosis and to provide treatment.
There are nearly 30 other diseases that can also mimic pheo. These are generally much rarer and I do not see them often. Finally, I want to emphasize that the above is based on my personal experience; other doctors may have a different view.
Dr. Pheo
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What a great blog, thank you for this. People do their own research and this is a great source of rich information.
ReplyDeleteI have a question for you... I have a SDHD gene mutation and I have every tumor known to have with that mutation. I also have an adrenal gland tumor and it was diagnosed a few years ago because before the SDHD gene mutation was found my doctor suspected I had this, and wanted to look at my adrenals.Low and behold, its there. When I first got the scan about 18 months ago they said its 8mm, I had another scan not to long ago and its 9mm. Its growing very slowly. Its not doing anything though, I have 24 hour pee tests done over and over again. Everything seems fine. I want to get this tumor out, because my body is "coded" for the tumor to become a pheo. Also, the state of our political enviroment is scary to me, and If I wait until this tumor starts acting up in 1 year, 5 years, 20 years, will, at that point, my Dr not not be able to because of healthcare laws and rationing. Also, I have two young kids at home and my husband travels for a living, and my tumor starts to act up, It would very hard for me to the surgery on short notice, it can be done, but very hard to get things together in short notice. I have had 10 surgeries, and on my way to having number 11 this fall, and it takes a huge amount of cordination on everybodys part to make it smooth. The next surgery I plan to have is to take a tumor out of my ear, and I have asked my ENT if I could have the surgery to remove my adrenal gland tumor at that time too, because my thoughts are if I am under, why not go ahead and do this too because I also dont handle anesthesia very well either. So, what are your thoughts on this? Thank you in advance. Heather
Dear Heather,
ReplyDeleteThe reasons to watch these small adrenal tumors are that they may grow slowly and we only have two adrenals. On the other hand, I understand your concerns of health care environment.
I will re-examine the adrenal mass on imaging to see if it indeed has the characteristics of a pheo. If it does, then resection is more indicated but you have to accept that it may not be a pheo. If on imaging, the mass has the characteristics of an adenoma, I will be reluctant to take it out. Partial adrenalectomy (with only the tumor taken out but not the good adrenal) is an ideal option you should consider although only some centers do it.
Dr. Pheo
Dr. Pheo, where are you located? I would love to come and see you. I am lucky enough to have a husband that is a pilot for a major airline, so getting in to see you for an appointment, even across country, is no big deal. You seem to know more about this stuff than the whole staff at Vandy in Nashville put together. BTW, I am in Orlando FL now. So even if I cant get in to see you, do you know anyone in FL that is as good as you?
ReplyDeleteThanks Heather
This comment has been removed by a blog administrator.
ReplyDeleteSorry, but what are the centers that do the Partial adrenalectomy? I promise to try to make these in one post next time.
ReplyDeleteHeather
Dear Heather,
ReplyDeleteThank you for the nice words. I removed your comment containing your email address to safeguard your privacy. Although I will be more than happy to see you, I suggest that you see Dr. Pacak at NIH instead. He is close to Florida and NIH has a large experience in partial adrenalectomy. Dr. Pacak is an outstanding doctor and does research in pheo genetics as well. He will help you decide whether the partial adrenalectomy is right for you.
Dr. Pheo
Thank you for removing my email address, I didnt even think of that. I guess that is why they pay you the big bucks, LOL. I found an email address for that DR at NIH, I will send him an email. Thanks for all the info, and I will be looking back often.
ReplyDeleteMaybe I am being silly about this whole thing,maybe I dont really have a pheo. I have never found a Doctor that knows about SDHD and what comes along with it. So, all I hear is the wait and see apporch because most people dont have pheos they just have a tumor. I want to get to the bottom of this to find out what is going on and to help me figure out my next move. It’s hard to take what Drs tell me at face value. I have never been told anything but let’s wait and see about my adrenal gland tumor. I once had a DR at a very large, world reendowed hospital tell me "Hon, people get tumors (I went to see her after my adrenal gland tumor was found and my other 5 tumors were found. I told her everything, did not leave anything out about my history.)There is nothing to worry about; I couldn’t count the people I see with all kinds of tumors." I then asked her if she felt like something else could be going on... maybe a genetic condition. She said "Nope, they just happen." Well, at that point I told her she was wrong, and a woman the age of 26 does not just get tumors? I left her office running and when I got the genetic diagnoses for SDHD, I sent her a letter explaining why I was not paying the bill they sent me, and she should learn to listen to her patients. I also sent her a copy of the genetic test with the positive results. This Doctor is an Endo Dr, and I will leave out what Hospital she works for. I am sure she is a good Doctor in other areas, or maybe she was in a bad mood having to work with residents, I am not sure. I am not telling you this to slam her up against the wall, because her expertise maybe with another part of Endo, but I try really hard to figure out what is going on with me, and to have someone just tell me (in not so many words, but this is what I felt) your just crazy. It’s hard to hear that sort of thing, during times when I need someone to help me through this. So with that, this is when I called the genetic clinic and made my own appointment. And that is what I do now; I make my own appointments and get my own answers. I always do my own research and bring those findings to the Doctor when I go. I also had a Doctor tell me to stop doing my own research, those websites can scare you. Well, I never went back to that Doctor, because he made me feel like I was not smart enough to navigate through medical terminology.
ReplyDeleteHe was halfway right because if you go to WebMD and type in a cut on your finger, up pops everything from wash it with soap and water to you have cancer and will die in two days. But, without my research I have done on my own, I don’t know where I would be. Plus, I would have not made it to your blog (trying to win brownie points, is it working?). This brings me to my next subject, Politics. Because of my private Healthcare that I currently have the privilege of having, I get to choose the Doctors I see. If I don’t like what one Doctor tells me, I pick and leave and I get a second, third, or fourth opinion with other Doctors. One day I fear that I do not have that option when everyone gets thrown into the Government run healthcare insurance program. Yes, everyone needs healthcare. There are people that can’t afford it, and its sad, and I do feel sorry for them. (See, I am not some right wing heartless nut case, I do have feelings.) But at what cost, do we give free healthcare to all and get stuck with a system like Canada’s where if a Doctor tells you one thing, don’t go looking for a second opinion because you will rock the boat and lose what you have there. I have heard first hand of that sort of thing happening, and I don’t like it.
ReplyDeleteDr. Pheo, I am so sorry for going on, and I invite you to take a look at my blog, confessions of a mad, mutant Mom. And I COMPLETLY understand if you delete this blog, I kinda hope you do. It has nothing to do with Pheos, but I have no way to contact you outside of the blog. Which is fine, I understand. Anyway, thanks for letting me rant and rave. Thanks for everything, I will be looking often, please keep up this very informative blog, you will touch many people. BTW, I will let you know what comes from my visit at NIH.
Heather
Dear Heather,
ReplyDeleteYou are welcome to share your experience and express your feelings at this blog. Keep me posted.
Dr. Pheo
Hi Dr Pheo
ReplyDeleteLet's say that someone tests negative for pheo (serum metanephrines) (I'm still waiting for mine!) and has a negative abdominal scan (at least that is out of the way although I realise it might be somewhere else!), by your excellent post the next step would be to investigate sleep apnoea and/or anxiety disorder.
How easy is it to decide that anxiety disorder is the cause? I've read about it (described as pseudopheo - is this the same thing?) I mean at what point do you say it isn't that and continue the search amongst the other 30 or so diseases?
I've also read that it tends to be in patients who repress or dissociate from the source of their anxiety. Is this your experience?
Hi Dr Pheo,
ReplyDeleteI have all the symptoms of a Pheo, and was diagnosed friday by an endocrinologist. The problem is my PCP and oncologist(seeing him for genetic testing-unrelated) told me the tumor I have is just a harmless adenoma according to ct scans and mri both with dye. My 24 hr urine test showed I tested high in norepinephrine that was 3x the norm and elevated epinephrine levels. I wasnt episodic when i did the test. I am also on clonidine 0.2 3x a day along with 100 mg metoprolol 2 x a day.
Is it possible the pheo is coming from somewhere else besides the tumor on my adrenal gland? Can they see from a mri and a ct scan if that the adrenal tumor is hormone producing? Or does that only show up in blood and urine?
Dear SH74130,
ReplyDeleteAlthough my personal experience is that sleep apnea and anxiety are more common, they are not necessarily the next steps for all patients. Your doctor has to evaluate you carefully and see what the most likely next disease it can be.
Dr. Pheo
Dear Theresa,
ReplyDeleteYour case can go both ways. Your doctors need to consider all info available to make two decisions: 1) whether you have pheo, 2) if you have one, where it is.
No imaging can tell if an adrenal tumor produces catecholamines or not. If the adrenal tumor appears as an adenoma, it decreases the chances that it is pheo but does not exclude the possibility.
I suggest that you test plasma metanephrines, followed by a clonidine suppression test (if the plasma metanephrines are abnormal).
Dr. Pheo
Dr Pheo, my 38 year old friend is from Oregon. She only has one adrenal gland due to a wilms tumor as an infant. She has been diagnosed with a pheo in her remaining adrenal gland based on 24 hr urine collection and imaging. She would like to make an apt to see you. How can she set that up? Thank You
ReplyDeleteDear shaynecorson,
ReplyDeleteThere is actually some association between Wilm's tumor and pheo. Your friend can see Dr. Dr. Paul Fitzgerald in San Francisco or Dr. Run Yu in Los Angeles, both reasonably close to Oregon. Any other doctors on Dr. Pheo's Pheo doctor list are also excellent.
What are your friend's specific questions she wants to ask me?
Dr. Pheo
Dear Dr. Pheo,
ReplyDeleteThank you so much for your quick response. I have been in a whirlwind with my emotions since I was diagnosed. I stumbled across your blog while doing research. Bless you for providing us with this information and your expertise! I think I may even start my own blog from a patients point of view!
There are several patient blogs out there. I have one: LifeWithPheo.Blogspot.com. There are a few more on Blogspot. If you would like the list of 4-5 I have, let me know.
ReplyDeletePam
Dr. Pheo, Thank you for responding. She has been advised that it isn't likely that her adrenal gland can be spared based on review of the scans. The tumor is about 3.8 cm and taking up most of the gland.The local endo here has little experience with pheo.
ReplyDeleteShe is very concerned about the prospect of life with no adrenal glands. She wants to find the best endo possible to first diagnose her and then treat her post surgery. Other than her adrenal tumor her health is excellent.
Do you have any specific thoughts on a Dr. who has had good results treating patients with no adrenal glands. She is able to travel anywhere in the US.
Are you aware of any other possible procedures that might be able to spare at least part of the gland.
Dear shaynecorson,
ReplyDeleteIt will be challenging to preserve even part of her remaining adrenal gland if the tumor has already taken up most of the gland.
Life without adrenal glands is not too bad. She will of course have adrenal insufficiency. She will need to take 1-3 medications for life and will have good quality of life. Most endocrinologists should be adequate in treating adrenal insufficiency. It is better to have a local endocrinologist to take care of her day-to-day needs.
I will post a new article on the pros and cons of seeing an out-of-town expert, especially me. Please ask her to review the article.
Dr. Pheo
Pam, thanks! I bookmarked it!
ReplyDeleteThis comment has been removed by a blog administrator.
ReplyDeleteDear shaynecorson,
ReplyDeleteI have sent you an email. I removed your last comment containing your email address to protect your privacy.
Dr. Pheo
Hello Dr. Pheo,
ReplyDeleteIs it possible to have a Pheo after having a Carcinoid tumor?
In 2007 I had an appendeceal carcinoid removed, during a surgery for endometriosis.
Felt much better after it was removed and my horrific symptoms resolved.
2008- tachycardia,anxiety,sweating, headaches returned. At the time I had abnormal plasma norieprinephrine,dopamine and seratonin levels but normal 24hr urine catecholmines. I even had an adrenal study CT, no tumors. Over the years symptoms would come and go but were not soo horrific...until I started to get chronic pancreatitis:(
Presently,after a thyroiditis attack, My symptoms have returned terribly. my episodic attacks: severe headache,vomiting,high blood pressure and tachycardia, red,flushed cheeks,cold,clammy excessive sweating,insomnia, then fatigue and I have livendo reticularis on my abdomen. Even simple vaccumming can trigger the sweating,nausea and tachycardia.I recently have had some 24hr urinary noriep/creat but nothing in my plasma. I have been having protein in my urine and I am having severe drops in my VIT D levels.
Can a person with history of carcinoid have a pheo too?
I am curious if you thought I should continue to persue a pheo dx or just let it go...
Dear empath4u,
ReplyDeleteYou can certainly have carcinoid then pheo, or vice versa, or simultaneously. Your test results, however, do not suggest that you have pheo.
How big was your appendix carcinoid?
Dr. Pheo
I just need some direction on where to turn next. Six years ago, I started having a racing heart and sweating. Then flushing of the face started. I don't feel it, and only know it happens when I look in a mirror or someone tells me. Then I had an octreoscan, which was positive somewhere in the ileum but they thought it was false and due to stool as I didn't do a bowel prep. I asked but was told it wasn't necessary. Apparently, it was but I digress. I have elevated serum serotonin about twice the normal limit from my lab. All other tests are normal. Next, I got a rash that has lasted about a year now and biopsies say one of the gyrates or connective tissue disease. Now hands are flushing bright red and paroxysmal hypertension is happening. I can be completely normal at 125/75 and a heart rate of 80 then spike to 180/110 or 180/125 with a rate of 115 or so. I also get pounding headaches and my vision isn't always what it should be. It just seems off. My docs have guessed mastocytosis, carcinoid and pheo but so far, everything is normal. Ideas on where to go next? I don't feel anxious other than I don't like worrying about what is going on physically.
ReplyDeleteDear skibo0803,
ReplyDeleteI feel your frustration. There are no simple solutions. At this time, I would see a cardiologist to get a 24-hour ambulatory blood pressure monitoring.
Dr. Pheo