Pheo is a fascinating but challenging disease. In the last 10 years,
we witnessed the explosive growth in pheo genetics and pathogenesis. Genetic
tests for pheo are now widely available. We also observed the unraveling of the
natural history of pheo and the understanding of pheo-induced heart problems. More
doctors now recognize that small pheos exhibit features distinct from those of
large pheos. The diagnosis of pheo progressed right in front of our eyes. Metanephrines
are now accepted by most doctors as the best tests and are more available world-wide.
The imaging characteristics of pheo were firmly established with the emergence
of many nuclear imaging tools. In spite of the advancements, two major challenges
still face us: misdiagnosis of pheo and management of malignant pheo.
I will continue the blog as long as I can. I will remain anonymous.
Dr. Pheo
Have my right adrenal removed due to a pheo in 2017. I am now have the same symptoms I had back then. Chills, palpitations, hot and cold at the same time. It was 10 years for doctors to figure what was wrong with me. The pheo was found in a scan I had for something else. Every once and while I would pass over when I stepped on a stool or a chair. No one could figure it out. I wore 24 hour heart monitors for palpitations. No one could figure it out. I have HBP which started around the time the incidentaloma was found. When I finally got the proper diagnosis it was because my CATS were 3 times their normal. I had the affected adrenal removed and attend all my follow-ups. I am wondering if it is possible to have the same issue on my other adrenal? Finished all the required labwork a few days ago, hoping my doctors are discussing the results right now. I haven't heard anything except that my thyroid was normal. I was hoping that removing the affected adrenal gland would be the end of this for me.
ReplyDeleteDear Anonymous,
DeletePheo recurrence or metastasis is a concern but it is unusual to have such quick recurrence of metastasis. Let's see what the test results will be.
Dr. Pheo
Congratulations on 10 years, Dr. Pheo!! You have been such a source of valuable information for those of us who suffer from this disease.
ReplyDeleteI hope you remember me and my family history of para/pheo. I am glad to say that my grandson, Xander, is doing well and there have been no more discoveries of pheo in any other family members, thank God.
On another front, I had open heart surgery in December of 2017 and developed quite a bit of scar tissue in my right breast for which they did MRI's to confirm...unfortunately, they found triple negative breast cancer in the other breast for which I had surgery, chemo and now have 2 more weeks of radiation. I am doing well, btw.
I seem to be taking it all in stride, though. After seeing the struggles here and in my own family, this is just one more bump in the journey of life.
I am eternally grateful for your help to us all on this blog. God bless.
Best,
Frances
Dear Francis,
ReplyDeleteOf course I remember you. Thank you very much for your staunch support in all these years!
Best wishes to everyone!
Dr. Pheo
Dear Dr. Pheo, thank you for your outstanding blog and also for your generosity and dedication to readers for the past 10 years. I have a 2.2 cm tumor in my left adrenal gland; plasma fractionated normetanephrine recently came back elevated (148). I've been having cardiac episodes for years (high bp, tachycardia, sweating, severe headache, one episode also with life-threatening a-fib), some of which have been serious enough to require hospitalization. I had the radiologist who recently identified the tumor go back and look at historical scans and he was able to find the tumor (smaller) as far back as 2009, though many radiologists between then and now missed it (I've had a lot of scans due to kidney problems). I am ready to say goodbye to this tumor. My current issue is that it appears on CT and MRI as more looking like an adenoma than a pheo (too fatty to be a pheo). What is the chance that it is a pheo that is appearing atypically on CT and MRI? If it's functional, and it certainly feels like it is, does it matter whether it's a pheo or not in a decision to remove it? Many thanks in advance for your kindness and help.
ReplyDeleteDear Anonymous,
DeleteIf the normal reference is normetanephrine is 0−145 pg/mL and your result is 148, you very unlikely have pheo. The imaging feature of the adrenal tumor also does not support pheo diagnosis. The pheo symptoms are not specific and can be caused by many other conditions.
Dr. Pheo
Thank you so much for replying so quickly, Dr. Pheo. I'm sorry I gave you a typo; plasma fractionated normetanephrine was 173 with the normal range ending at 148. That's what I meant to say, and it's up from 87 measured three years ago. I'm looking forward to hearing what you have to say next. Kind regards.
ReplyDeleteAlso, it's my understanding from this study that many people with smaller pheos have normal or slightly elevated norepinephrine. Would you concur? https://onlinelibrary.wiley.com/doi/full/10.1111/j.1751-7176.2012.00604.x
ReplyDeleteI meant normetanephrine, as measured in fractionated plasma metanephrine. Some people with smaller pheos seem to have normal or slightly elevated normetanephrine, according to this study.
ReplyDeleteDear Anonymous,
DeleteI am aware of that study. Most people with small pheos don't have pheo symptoms. Normetanephrine levels fluctuate with stress levels. I still think you don't have pheo.
Dr. Pheo
Hi D.E. Pheo...
ReplyDeleteI just heard about your blog. I would love any input.
03.18.19: I went to the Emergency Room for, unusual for me, bp at home and feeling of being unwell. Although I had a diagnosis of hypertension for a long time, it was very responsive to medication and while medicated never over 125/90. That day it was 143/97, 163/102, 156/106.
03.29.19: previously scheduled yearly checkup. I had been doing very well for a year, until the time around the ER visit. Noticing uptake in symptoms that happened in the month-Muscle Pain and Stiffness, muscle cramps, increased fatigue, overall weakness, increased heart rate-not correlated with increased BP we’re discussed at my appt.
Labs show Potassium had decreased again-Oral RX VitK up from 20 MeQ to 30.
05.30.19: I was feeling MUCH worse with new symptoms. Because I had a two-year history of Hypokalemia, I began to research causes and wondered the possibility of my newly profuse sweating, making me lose MORE potassium and feel worse. In 2016 I got Rocky Mountain Spotted Fever and much of my issues have been blamed, although I had improved to 70% the prior year.
By now, Worsening Fatigue, Weakness, “Sluggish Gut”, Profuse Sweating, stomach pressure, nausea, more muscle cramps in back, hips and feet, swelling and tingling of face and hands, sporadic head tingling and throbbing, Tachycardia-around or over 100-130.
Labs: Magnesium low- started on Max-oxide.
Cortisol was low-normal, and he felt I should then see an Endocrinologist and made a referral. Potassium was 4.0.
06.11.19-Endocrinology: She decided to start slow with a 24-hour Urine Cortisol, B12 and Vitamin. Vitamin D was low at 18, B12 was normal and the 24-hour urine was mixed up at the lab and results were discarded.
06.17.19: I returned to Internist after hard weekend. I pleaded with him to help me. Over the weekend I had a 4-day migraine, blood pressure was sort of all over the place during headache and 150/110 in his office. He looked up past and present rate and it correlated that my pattern had changed. He then suspected a Carcinoid and ordered 24-hour Urine for 5HAII, Catecholamines, Metanephrines, and repeated Cortisol. He also ran 8 labs, including magnesium, BMP, Testosterone, Estrogen, Prolactin, aldosterone, HA1c.
When I came home and researched the reasons for those urine tests and ran across pheo/para, they sounded just like me.
Abnormal Results:
Elevated HA1c-6.6 (gl143),
Elevated Estrogen of 75 (had full hysterectomy including ovaries in 2017),
Low than before Magnesium of 1.5
Increased Normetanephrines of 1025 (NL 119-451), Increased Metanephrines 1142 (NL 119-561), Increased Catecholine-Norepinephrine 128 (NL 15-80)
06.25.19:
Referred to endo/onc/surgery for the rest of the workup. I saw him Friday. He repeated aldosterone, added a renin and I have CT scans this week.
He seemed to be leading towards the aldosterone being an issue even though it was normal. Do you see anything that makes you think pheo/para is the wrong way to go or we should be adding other things during the workup?
Thank you!
Dear SparkleTrail,
DeleteHyperaldosteronism is a concern and should be examined. Pheo is also possible. Did you also measure 24-hour urine creatinine as well?
Dr. Pheo
Repeat aldosterone and renin were normal. Creatinine was normal on both 24 hour urine and my second one had almost identical numbers to the ones above.
DeleteI did have a CT and have the report but won’t be talking to my local doc about it until Friday. No abnormalities was found in my adrenal glands but these other things were found and have me wondering about paras. My doc has only treated one pheo before and I’m guessing the radiologists may not have seen them much either. I’m not comfortable with worlds like “probably” thought.
And I have not have any prior kinds of infections to explain any of these findings. No pneumonia’s or gramatulous history.
“Lower chest: A wedge-shaped peripheral opacity anteriorly in the left lingula measuring about 2.5 cm is likely scar or atelectasis.
A small 11 x 6 mm oval density posterior to the left atrium on the most superior axial image is probably calcification related to a lymph node.
Liver/biliary: Diffuse low-attenuation liver consistent with fatty infiltration. No gross hepatomegaly. No focal hepatic mass. Patent portal vein. No calcified gallstone or biliary dilatation.
Spleen: Normal in size without mass. Punctate calcifications compatible with prior granulomatous disease. Small splenule noted anteriorly.”
The rest was unremarkable, including adrenals.
Further thoughts? I may try to get to NIH since I can’t get local care even though I am local to UNC and DUke.
Thanks again for all you do.
Dear SparkleTrail,
DeleteThe urine metanephrines need to be corrected by urine creatinine. If after correction by creatinine, the urine metanephrines are still elevated, a paraganglimoa needs to be considered. You can do a Ga68-DOTATATE PET to look for a paraganglioma.
Dr. Pheo
Do healthy adrenals ever show intense, abnormal focal uptake on MIBG scans? Can MIBG scans have false positives?
ReplyDeleteDear Anonymous,
ReplyDeleteNormal adrenals should not show intense MIBG uptake. On the other hand, if the CT or MRI did not show an adrenal mass, having intense MIBG uptake itself should not be problem itself.
Dr. Pheo
What if they aren't "normal" adrenal glands? and even though it "should not be a problem" could it be????
ReplyDeleteDear Anonymous,
DeleteIt is a complicated issue. A small pheo or adrenal medulla hyperplasia can show strong MIBG uptake. Some patients and doctors report that small pheo and adrenal medulla hyperplasia can cause symptoms mimicking pheo. In my own experience, they don't cause clear problems.
Dr. Pheo
I have a small left adrenal mass that I've known about for several years. It will go a few years without changing in size and then suddenly it will have grown a bit. Last time the size increased, it did so by just under half a centimeter, but it's been stable for the last 2 years (it does appear to be a fatty adenoma on MRI). The endocrinologist continues to monitor it because of the size change, mildly elevated metanephrines several times, and because I had hyperparathyroidism in my 20's. Anyway, as part of my yearly bloodwork, I do a dexamethasone supression test, along with aldosterone and renin. Twice now, my aldosterone and renin have been high when it is checked the same day as cortisol (so morning after dexamethasone). The doctor says a 1mg dose should have no impact on aldosterone or renin, but if anything, would make them lower. That does not seem to be the case for me. Last time my aldosterone was 56 and renin was 5.62 after the dexamethasone. When they were re-tested a few days later, aldosterone was 13 and renin was 1.46. Results from the year before were very similar. I can't find anything online about a steroid causing elevated aldosterone or renin (I have seen things about steroids causing high blood pressure). The endocrinologist can't seem to explain why it happens either. I do have autonomic dysfunction (POTS, erythromelalgia, digestive issues) and idiopathic hypovolemia. Could that have anything to do with it? Is it likely totally unrelated to the adrenal mass? Any insight would be appreciated. Thank you very much.
ReplyDeleteDear Anonymous,
DeleteI am sorry but I also cannot find any literature on dexamethasone increasing renin and aldosterone. Renal artery stenosis may alter the dexamethasone effects on renin and aldosterone--this is just my surmise.
Dr. Pheo
Thank you for responding. That's interesting - my grandmother had renal artery stenosis, but I assume that would have shown up on MRI by now if it was an issue for me. I'll have to read more about it. We'll see how the bloodwork turns out this time around. Thanks again.
DeleteHello, physician here. Have you come across this article https://www.cnn.com/2019/07/27/health/doug-lindsay-invented-surgery-trnd/index.html ?
ReplyDeleteMy initial impression is that either the writer completely misunderstood the subject's history, or the subject is self-misdiagnosed or a Munchausen. Excessive adrenal medullary activity would not seem to cause the symptoms described, and I don't see how treatment with norepinephrine could be effective.
What do you think?
Dear zzlangerhans,
ReplyDeleteI just read the article. Please see my post on "adrenal medulla hyperplasia" on Jan 1, 2010 in this blog. I don't think adrenal medulla hyperplasia causes his symptoms or removal of adrenal medulla would be beneficial for him.
Dr. Pheo
Thanks, that's what I figured. It's fascinating how mass media always gravitates to skepticism of the medical establishment and the scientific method, suggesting that lay people would be better off perusing the internet for answers than listening to doctors. Kind of like that "doctors don't try to cure cancer because then they'd be out of a job" theory that seems to keep popping up.
ReplyDeleteI was wondering - have you ever seen a case where a patient has reacted to tyramine and beta blockers as if they had pheo, but no pheo could be found? I began having very classic pheo-like spells 5 years ago (racing heart, sweating, severe headache, hypertensive crises, tachycardia). Plasma and urine metanephrines intermittently elevated (I've had about 5 tests slightly high - maybe 2x upper limit of normal - and 5 tests well within normal range). Then three years after these spells started, I began reacting to tyramine. Even small amounts of tyramine cause a spell, including hypertensive crisis. If I test metanephrines after ingestion of tyramine, they are very high. I am not, nor have I ever been, on MAOIs; in fact, I was not on any medications when this started. I have had a Dotatate PET scan, several CT scans of the head, chest, and abdomen, as well as MRI of the chest, abdomen, pelvis, and head. I've had ultrasounds of the carotid arteries and neck due to suspicion of possible carotid body tumor. Nothing was ever found on any of these scans. Additionally, beta blockers cause extreme hypertension. Many other disorders, including psychiatric, have been thoroughly ruled out. My doctors are extremely confused as to what could be causing pheo-like spells, along with a pheo-like reaction to beta blockers, and such an extreme reaction to tyramine, if it isn't pheo. Have you ever seen tyramine reactions like this outside the context of pheo?
ReplyDeleteDear Anonymous,
DeleteI have not seen a patient with exactly your symptoms but I have seen patients with less severe hypertension after taking tyramine without MAOI. You may have a naturally weak MAO.
Dr. Pheo
Hi Dr. Pheo,
ReplyDeleteA new case of GIST has been found in my niece who has SDHB. Her half brother had 2 pheos and a Pulmonary chondroma. Is it unusual for a family have so many different tumors associated with SDHB? (pheo, para, neuroblastoma, gist and the chondroma). Makes me more than a little nervous.
Thank you as always,
Frances
Dear frances,
DeleteIt is indeed a concerning new development. On the other hand, all these tumors are known to be associated with SDHB mutation. Please make sure to have all affected family members to undergo appropriate surveillance.
With best regards,
Dr. Pheo
I am not sure where you're getting your info, but great topic. I needs to spend some time learning more or understanding more. Thanks for magnificent information I was looking for this info for my mission.
ReplyDeletedystonia treatment in homeopathy
Hi Doctor Pheo, I have a 17mm nodule. I have episodes of night time hyperactivity where I figit and can't sleep. Often I will go work in my shop until it subsides, usually 4-5 hours. I also have high blood pressure and fast heartbeats with these episodes. The Edos around here are mostly about diabetes. The one I have seen said just keep a watch for growth. I am willing to travel for a good diagnosis. I am in Amarillo Texas, Would MD Anderson in Houston be a good choice? Or I would welcome a recommendation. Thank you for your blog
ReplyDeleteDear robjohn,
DeleteAn adrenal tumor this small usually does not cause pheo symptoms. The imaging characteristics of the adrenal tumor is very helpful in determining the nature of this tumor. MD Anderson is a great place to get help.
Dr. Pheo
Dr. Pheo,
ReplyDeleteMy father had his right adrenal gland removed after doctors identified a 4cm tumor (it was not cancerous). His surgery occurred at the end of 2018. Over the past few months, he's had reoccurring episodes of a fast heart beat, high blood pressure, a strong pulsing sound in his ears, and significant shaking. Each time he goes to the doctor, they take a blood and urine sample and say that everything looks fine. So, there has been no further testing. In addition, during one episode he had shortness of breath, so he went to the ER. He was admitted for tests on his heart, and they said everything looked good.
The doctors that ran the tests include his primary care doctor, and the doctor that performed his surgery. Neither doctor has the considerable expertise/experience of the doctors on your list. And we are unclear how much experience they have with pheo.
We are trying to figure out whether to trust the blood and urine tests, or escalate the situation, perhaps by visiting someone on your list. My father lives near Chicago.
Dear JC,
ReplyDeleteYou can ask his doctor to order a test called "plasma metanephrines". If that is already done and normal, pheo is ruled out. If not done yet, it is the best test to see if he has pheo.
Dr. Pheo
Hi Dr Pheo
ReplyDeleteCan you please help? Small tumor found on adrenals have had the barrage of tests all came back normal with exception to Vit D and Iron. I also have Polycythemia Rubra Vera (not hereditary / gene related) and have always been kept anaemic due to PRV.
Anywho Renin was slightly elevated.
Got supine plasma metanephrine done and today got the results but don’t know what they exactly mean -
My metanephrine looks to be in the cut off range or reference range is pmol/L <300 and mine was 150.
My normetanephrine was pmol/L <660 reference but mine came in at 1720. Any views or advice? The bottom of the form says Pheo cannot be excluded.
P.S Anonymous again with the blood query I also had 24 hr urine done as well - umol / D < 3.00 my normetanephrine was 5.17 not sure if that makes any difference for the query about the blood test!
ReplyDeleteDear Anonymous,
DeleteWhat is the size of your adrenal tumor and how did the radiological report describe your tumor? Did the report say it is consistent with pheo?
Dr. Pheo
Hi Dr Pheo
ReplyDeleteI am 30 y/o. Have had PRV diagnosis since 8.
CT scan says: “nodule at right adrenal gland < 3CM that doesn’t show heterogenous solid or cystic pattern. Density of 78 on non-contrast. Nodule has mean density of 145 hounsfield unit on arterial scan. Marked enhancement on arterial and mean density on arterial scan of greater than 120 hounsfield units can be seen in cases of Pheo. Recommend urinary catecholamine levels is requested to rule out Pheo”
Cue the urinary test, followed by the supine plasma metanephrine tests. Thank you in advance and for taking the time to respond, it’s appreciated. I have found this blog a wonderful read, thanks for continuing the work you do here.
Dear Anonymous,
DeletePheo risk is substantial. Let's see what the test results will be. Do you know what mutation you have that causes the polycythemia?
Dr. Pheo
This comment has been removed by a blog administrator.
ReplyDelete