Patients with pheo often ask “why
do I have pheo?” I usually assure them that nothing we know of, other than
genetic risks, causes pheo. There is no clear evidence that common risk factors
such as smoking, obesity, diet, infection, and exposure to environmental toxins
are associated with higher pheo risk. There is also no clear evidence that
other diseases the patient has, other than those that are part of a genetic
syndrome, are associated with higher pheo risk.
An interesting paper published
online last month seems to suggest that cyanotic congenital heart diseases may
predispose people to develop pheo. The idea that hypoxia may lead to pheo is
not too new now. About half of the genes that cause pheos when mutated are
involved in making molecules carrying energy. As making energy molecules
requires oxygen, the mutated genes mislead the cell into thinking that it lacks
oxygen supply (hypoxia). The cellular processes that try to cope with hypoxia,
over time, can result in pheos. It has been reported that people living in high
altitude (thus low oxygen levels) tend to have more neck paras and perhaps pheos.
The new paper now shows that people with cyanotic congenital heart disease may also
tend to have more pheos.
Cyanotic congenital heart
disease is a congenital condition in which the abnormal heart structure causes
mixing of blood from the two sides of heart, thus low oxygen levels in the
artery and tissue. The new paper shows that people with cyanotic congenital
heart disease have a 6-fold risk of having a pheo diagnosis, compared with
those without, during their hospitalization for whatever causes. In the same
paper, the authors also identified 20 patients with congenital heart disease
and pheo, 18 of them having cyanotic disease, 2 having aortic coarctation. The
18 patients with cyanotic congenital heart disease were younger than the average
patients with pheo and tend to have multiple tumors. Unfortunately, the paper
does not specify the 18 patients were out of how many patients with cyanotic
congenital heart disease so that we don’t know the incidence of pheo in those
patients.
As the authors prudently
cautioned, their study only establishes an association between pheo and cyanotic
congenital heart disease but not a causal effect of chronic cyanosis. One other
potential bias is from ascertainment. If more patients with cyanotic congenital
heart disease are tested for pheo, more pheos will be diagnosed. Nonetheless, I
will ask my next patient with suspected pheo whether she/he has a history of
congenital heart disease, especially a cyanotic one.
Dr. Pheo
Hi there, was recently introduced to this forum.
ReplyDeleteI am a 29 year old male from Mumbai been diagnosed with hypertension since the age of 19. Often had BP spikes which were hard to control even with several medications (there was a period when I was on 4 different medications for BP) and have had periods when it is absolutely normal. Currently on Minipress (Prazocin Hydrochloride - 5 mg twice a day) and Telmisratan 80 mg for the HTN. Had my urine VMA tested several years back and plasma epinephrine and norepinephrine tests done which came back negative and hence pheo was ruled out (no test for metanephrine or normetanephrine was ordered). Other pheo-like symptoms included severe headaches, excessive sweating, anxiety attacks. Also get frequent nosebleeds, episodic bronchitis (asthma-like attacks), and experience "flushing" (face gets tanned in stressful situations).
Also had other tests to rule out renal hypertension including angio (renal MRI had shown stenosis), captopril scan etc. My 2D echo was fine, only with the indication of concentric LVH with EF of 60% and AML Prolapse into LA.
Had given up and accepted "essential hypertension" as a diagnosis. However, given persistent symptoms, recently visited a new doctor who started evaluation of carcinoid as a possible diagnosis (though he too suspected pheo given the symptoms but decided to pursue carcinoid since it was not explored earlier). Ordered 5-hiaa urine which came slightly elevated at 10.4 mg/24 hrs (ref range: 2-6 mg/24 hrs), CgA levels also came elevated at 145 ng/ml (normal < 100 ng/ml). I just underwent my Ga-68 DOTA NOC scan which came back negative for any abnormal receptor uptake. However, the only abnormalities were some bilateral maxillary polyps and some soft tissue density nodule in apicoposterior segment of upper lobe of left lung ("likely to be non-specific"). Also, focus of calcification was seen in prevascular lymph node.
Just wanted to check what this could imply? Could it still be carcinoid? Could it be Pheo and further testing could be explored since symptoms match? Or could it be another NET? Or is it nothing at all?
I would deeply appreciate any help I could get from you.
Best regards,
Varun
Dear Varun,
ReplyDeleteOverall the probability of pheo or carcinoid is low. When those tumors produce symptoms as you already have, the tumors are usually not subtle but rather sizable with markedly elevated tumor marker levels.
Dr. Pheo
Dear Dr. Pheo,
ReplyDeleteI’m a 30 year old woman, 5’1, 115 lbs, who's had intermittent microhematuria for the past 8 years, and intermittent hypertension for at least the past 4. Since 2011, I’ve had random spells that involve spikes in blood pressure, dizziness, blurry vision, and sometimes (but not always) double vision and hot flashes, and nausea. These spells are usually precipitated by external stressors, but when I began having them in 2011, they were not at all related to stress. Sometimes when I wake up in the morning, I am nauseous and/or I have very bad periorbital edema on one or both sides of my face (sometimes my eye is nearly swollen shut) that sometimes goes down throughout the day, but other times lasts for days. I also have intermittent joint pain (in mostly my knees, but sometimes in my hips and shoulders). For the past year or so, I've been getting headaches with pain behind both of my eyes. Recently, after acquiring a cold that developed into a sinus infection (almost all my colds do), my blood pressure spiked to 145/108, and was high (averaging around 140/104) for the last few weeks, but then dropped to 120/95 three days ago.
Lupus, Sjogren’s, Ehlers-Danlos Syndrome, Lyme Disease, Hepatitis (B and C), and Fibromuscular Dysplasia have all been ruled out. My C3 and C4 levels are normal, and my creatinine clearance has decreased over the years, but my eGFR and my creatinine clearances are still just within the safe range, despite the macrohematuria and hypertension.
I also have PCOS, and all the classic symptoms of it except for hair loss (which only occurs when I take Spironolactone/Aldactone) and except for overweight. This makes me think maybe I have a pheochromocytoma, or some kind of either adrenal or pituitary problem. The cardiologist I last saw seemed to think that, too.
Is it possible to see macrohematuria and intermittent hypertension with pheochromocytomas? Or could I have a pituitary tumor?
**My thyroid levels were all normal, as well.
Delete**Also, my electrocardiogram looked fine, and my ultrasounds of my arteries and kidneys all look okay also.
ReplyDeleteDear Anonymous,
DeleteA bladder paraganglioma (similar to pheo) can present with hematuria and hypertension. You can test whether you have it by plasma metanephrines.
Dr. Pheo
Dr. Pheo,
ReplyDeleteGlad to see you are keeping this blog active- I am so glad to have found it. I have POTS and recently underwent a plethora of testing at the Cleveland Clinic in an attempt to find the underlying cause as some symptoms have become increasingly severe. My neurologist ran a urine metanephrine test and my levels came back elevated with metanehrines at 859mcg/24hr and normetaneprines at 1615mcg/24hr. I've been reading that levels that are one to two times the normal range *might* be a pheo, but levels more than double are quite likely to be a pheo. My question is about the normal range. Attached to my lab results, the normal range show the upper limit for metanephrine as 399mcg/24hr and 899mcg/24hr for normetanephrine- which means I am not doubled. However, the few sources I've seen online show much lower upper limits- 180mcg/24hr for metanephrines and 484mcg/24hr or even a bit lower. In those cases- I'm much more than doubled. The numbers seem vastly different- could you possibly shed some light on them for me?
Dear Elizabeth,
DeleteThis is a common issue for pheo test results interpretation. The labs use different methods in measuring metanephrines and other pheo markers so that the reference ranges are different. The results should be interpreted based on the specific reference range of a lab.
Dr. Pheo
Thank you. I saw an endocrinologist today through a major university medical center, and just my luck she said she has never seen my presentation of test results before. She said often she will see high metanephrines in the blood test but normal levels in the urine, which indicate a false positive in the blood test. My results were the opposite- very elevated urine but normal blood levels. She didn't know what to make of it, so she is repeating the 24 hour urine test. Is this something you have ever encountered before?
DeleteDear Elizabeth,
DeleteThe blood and urine tests are both good if interpreted correctly. Any negative metanephrine results rule out pheo, whether they are blood or urine. Thus in your case, the probability of pheo is very low.
Dr. Pheo
As new drugs come to market, some are coming now with black box warnings like Victoza for MTC based on animal studies. Are there any medicines today which should have one for pheo... such as Accutane? (Which is not longer prescribed like aspirin when it was first introduced) Or is it that the Fischer rats used are over-breed and are predisposed to tumors and drugs like accutane may push a person to develop pheos?
ReplyDeleteDear Anonymous,
DeleteThe Fisher 344 rats are prone to endocrine tumors. They are often used as models of endocrine tumors. I am not aware of any human studies suggesting accutane may be associated with pheo.
Dr. Pheo
Dear Dr Pheo, can you please advise how much pheochromocytoma generally grow each year? I've been having many symptoms for the past 9 months (since July) such as postural tachycardia, heavy pounding heartbeat, heartbeat in ears, sporadic high blood pressure, sweating and hot flashes, nausea, breathlessness on exertion, intolerance to medications especially antidepressants (which I have only been prescribed due to my symptoms) and metaclopramide, shakes especially in the morning, severe thirst but I urinate continuously so never feel hydrated. There are many other symptoms too. I had a general CT of my abdomen and pelvis in October which did not show anything. Can a pheochromocytoma be missed on a CT scan? My symptoms are becoming so severe now I barely leave the house. I have had many bloods taken and my blood count, glucose etc are all normal. I am currently awaiting the results of a 24hr acid urine test, but I'm wondering if I did have pheochromocytoma surely it would have shown up on the CT scan which was only 5 months ago? The other thing I wanted to mention was that the heavy pounding heartbeat breathlessness and sporadic nausea began 9years ago during pregnancy and has never gone, but it wasn't severe enough to cause daily struggles. I also have severe upper abdominal bloating and discomfort with pressure and fullness around my rib cage especially the right rib, along with back pain and flank pain. Is it possible to have a pheochromocytoma undiagnosed for 9 years? I was wondering if maybe a small pheochromocytoma can begin to grow but stop growing for many years, or is a paraganglioma possible? If the urine test does come back positive I'm not sure if only my abdomen will be scanned or my whole body? The k you for your time. Mel
ReplyDeleteSorry but I also forgot to mention that I have also lost 2 stone in weight. I am 5ft 5' and now weight 7st 6lb. Another symptom I have is vision distortion at the side of my vision when against a light background, along with being very sensitive to daylight. Thank you.
DeleteDear Melanie,
DeletePheo usually grows slowly (a few millimeters a year). Most functional pheos are in the abdomen and pelvis but occasionally they can be at other places such as the heart. If the pheo markers are normal, then pheo is ruled out. If the markers are clearly elevated, then CT or MRI of the neck and chest is needed.
Dr. Pheo
Thank you very much for your reply.
DeleteIf Catecholamines tests continuously show elevated dopamine levels - what does this mean? I am experiencing all symptoms that would be experienced with pheo yet plasma methephrines came back normal. Nothing makes sense ... But continuing feeling nausea and vomitting, burning pain in back and pain in flank area... And so tired.
ReplyDeleteThis comment has been removed by the author.
DeleteI should have also said suffer from hypertension, loss of appetite and weight loss. Further tests being carried out due to hemauria .. So testing for protein.
ReplyDeleteDr Pheo, I was diagnosed with hypertension 17 years ago at the age of 32. My kidneys checked out fine. My blood pressure was controlled until two years ago when I started having blood pressure spikes of 190-200/110. With the blood pressure spikes comes a terrible headache, facial flushing sweating, anxiousness/jittery and heart palpitations. Exercise bring these episodes on and they last for hours. (I have gained 30 pounds in 6-8 months). I was cleared of carcinoid syndrome, but have yet to have a diagnosis. Does this sound like a pheo? If not, do you have any suggestions on test/bloodwork I should request? I have seen so many doctors and just keep getting worse. Please help me.
ReplyDeleteDr Pheo,
ReplyDeleteCan I also ask if drinking too much fluid can affect the 24hr acid urine test results, making them inaccurate? When I did my test I drank at least 3 litres of fluid due to excessive thirst, and I'm now concerned this may affect the results. Thank you for your time.
Dr Pheo,
ReplyDeleteCan I also ask if drinking too much fluid can affect the 24hr acid urine test results, making them inaccurate? When I did my test I drank at least 3 litres of fluid due to excessive thirst, and I'm now concerned this may affect the results. Thank you for your time.
Dear Melanie,
DeleteIf the marker levels are corrected by creatinine, urine volume does not matter.
Dr. Pheo
Dr Pheo,
ReplyDeleteThank you so much for your informative blog. The information you provided helped me greatly with the care of my husband. He was recently diagnosed with a pheo, underwent laparoscopic removal and is recovering. Quite a journey.
I read your recommendations concerning follow-up posted in 2009. Are they still current?
Again, thank you for having this blog. The information that you provide makes it easier to navigate the "medical industrial complex" that I was a part of many years ago.
Dear Anonymous,
DeleteThe follow-up of sporadic pheo is still the same.
Dr. Pheo
Dear, Dr. pheo
ReplyDeleteI'd like to get your advice please.
3 weeks ago i was diagnosed with with pheo.
It started about a month ago when i went to the hospital with symptoms of vomiting, elevated heart rate and elevated blood pressure, with anxiety, headache, hypokalemia, and elevated sugar.
It turns out that i had a slight heart attack with a positive cardiac enzyme test. my troponin levels were slightly positive. To lower my bp immediately, they gave me a nitrostat under my tongue and an IV dose of lopresser. They released me after 3 days, still i was not diagnosed with pheo.
7 days later i had a 2nd attack with the same symptoms. i went back to the hospital after during a 24 hour urine collection which took one week for the results to come back along with a blood test.
They diagnosed me with pheo and a 7cm tumor on my right adrenal.
The first general surgeons said they wanted to remove the whole adrenal gland because they say it is most likely too intertwined and they can't tell which is the gland and which is the tumor.
They gave me a 3 day supply of phenoxl benzamine and a script for 30 day supply. I wasn't able to get the phenoxl benzamine due to the cost, so they put me on prazosin 1mg, 2 capsules. twice/day.
Then 7 days later again, for the 3rd time, i had the same symptoms. i went back to the hospital. they gave me metaformin, insulin, prazosin and xanax.
I've found a specialist instead, an endocrine surgeon by the name of Dr. David N. Bimston, out of Hollywood FL, on May 4th.
Have you heard of Dr Bimston of of Hollywood, FL?
I read you recommend Dr Larry Kvols. He is at least 4 hours away and I am not sure if I can get there but I would consider it.
How many pheo operations should I expect a surgeon to have performed to be more of an expert at it?
Thank you for any help.
Jason
Dear Jason,
DeleteI don't know Dr. Bimston. Dr Kvols may have retired but you many want to verify. Dr Kvols is not a surgeon himself. I would consider a surgeon to be reasonably experienced if she/he has performed 5-10 pheo surgeries.
Dr. Pheo
Dr Pheo,
ReplyDeleteI was diagnosed with an adrenal adenoma in 2006. Between 2006 and 2009, I had 2 MRI's - no change. I also had high calcium starting in 2006 and had one parathyroid gland removed in 2013. Periodically over the years, I have had episodes of high blood pressure, high heart rate, headache, and stomach pain. In 2010, my urinary metanephrines were slightly elevated (215 - normal was 90-140). Before parathyroid surgery, my plasma metanephrines were 51 (58 was high end of normal). Starting about 3 weeks ago, I had terrible insomnia (2 hrs or less of sleep per night), palpitations, nausea, headaches, and trembling. It got so bad, I went to the ER. BP was 170/110. Not sure what my heart rate was, but it was high enough for them to keep me overnight to monitor. Plasma metanephrines came back at 63 (so, only slightly elevated). Urinary metanephrines also slightly elevated (195 - high end of normal was 190). Repeat MRI showed increase in size of adenoma by .4cm (it is still under 2 cm in size). No sleep aids helped the insomnia, but Lorazepam has allowed me to sleep 5-6 hours per night for the last week or so. Waiting to see my endocrinologist, but wondering what the best course of action is? Retest plasma metanephrines? Would the Lorazepam impact those results? Thank you.
I am 35 years old now - sorry, meant to include that in my original post, in case it matters.
DeleteDear Joyce,
ReplyDeleteYour age is actually a very important factor here. It is very rare for people under 40 to have primary hyperparathyroidism or adrenal adenoma. Are your calcium levels normal now? How much are your parathyroid hormone levels now? What did the MRI report exactly say? Have you done tests to see if the adrenal tumor is functioning? The above information is important in determining whether the parathyroid tumor and adrenal tumor are related. Your symptoms may or may not be related to the adrenal tumor.
I've had both the high calcium and adrenal adenoma since I was 26. My calcium levels are normal now, but seem to be on the increase. For the first 6 months after surgery, they were in the low 9's. For the last 6-8 months, they have ranged from 9.8 - 10.5. PTH has been normal - the highest so far has been 44 (lowest was 30). During surgery my PTH was almost 1100 and went down to 19 within 10 minutes, so they considered me cured. Oddly enough, I only had truly high PTH once since the high calcium was discovered, otherwise it was always at the high end of normal. My calcium always stayed in the low 11's. Anyway, they removed the lower right gland and said it was "very large."
ReplyDeleteThe most recent MRI says "there is a 1.5 x .7cm left adrenal nodule (previously 1.1cm x.7cm). This demonstrates loss of signal on opposed phase images although is less conspicuous due to paucity of intra-abdominal fat. There is peripheral enhancement following contrast administration. Two subcentimeter hyperintense lesions are seen within the left kidney, to small to accurately characterize. My other 2 MRI's said the size was unchanged, so this is first time there's been any increase.
Plasma metanephrines came back slightly elevated at 63 (high end of normal is 58). I've had the urinary metanephrines checked twice - one came back at 215 - normal cut off was 140. I had it checked again recently and it came back at 195.
The surgeon who removed my parathyroid commented on how unusual it was to have both at a young age, but at the time, no one in my family had either problem. They did recently discover an adenoma on my grandmother's adrenal gland and she does have high blood pressure and episodes of tachycardia. She is currently undergoing testing - I do know that her coritsol was high so far, but haven't heard the results of anything else.
I'm on Lorazepam currently for sleep (I could not sleep more than 2-3 hours a night and was having frequent episodes of high bp, tachycardia, whole body trembling, and headaches). If they retest plasma metanephrines, I assume I will need to stop the Lorazepam. I realize I may be totally wrong, but I feel like the Lorazepam is the only thing keeping me from having those spells.
I apologize for the length of this response, it was much longer than I intended. Thank you.
Dear Joyce,
DeleteThis is a challenging situation. There is some suggestion that you may have MEN2 with de novo mutation but the evidence is not strong enough. I would continue follow the calcium, PTH, and metanephrine levels periodically. Taking lorazepam should not significantly interfere with the pheo testing.
Dr. Pheo
This comment has been removed by the author.
DeleteHi Dr. Pheo,
DeleteI have a jug to start the 24 hour urine collection after my next big episode. S, we'll see how that turns out. In the meantime, my endocrinologist has tested my aldosterone, rennin, acth, and cortisol. Aldosterone was high, renin was normal, acth was normal, and cortisole was slightly over the normal limit. Is it odd to have slightly elevated plasm metanephrines, high aldosterone, and slightly elevated cortisol all at the same time? My blood sugar is also on the rise, which is something I've never had a problem with before.
Thank you,
Joyce
Anonymous said...
ReplyDeleteDr. Pheo, I had a 4.0 cm tumor removed in May of 2014 due to a pheo tumor on my left adrenal gland. I do NOT have high blood pressure now that the tumor is removed, but I do have severe headaches that never end. I also have had genetic testing and have found to have a 5-MTHFTHR gene with a TT. My family physician says it was inherited from my mother's side of the family, but both my mother and father each had the TT gene and passed it on to me. Please, can you help me with the never-ending headaches I suffer from. I can hardly stand the pain.
April 30, 2015 at 5:56 PM
Please prove you're not a robot
Hi Dr Pheo.
ReplyDeleteI am just wondering how easy it is to tell a single para apart from one that has infiltrated a lymph node. I have multiple paras that show up on MRI and Gallium 68 PET. Can they tell if the paras are not in lymph just by looking at scans?
Many thanks
Dear Simone,
DeleteIt is hard and sometimes impossible to tell. If the tumor is in the usual place for a para (along the spine), then more likely it is a primary para. If it is in a place where para should not emerge, then more likely it is a lymph node.
Dr. Pheo
Hi Dr. Pheo, I did a 24 hour urine metanephrine test. During the day of collection, the collection container was stored in a malfunctioning fridge that was warmer than a normal fridge (maybe 8-10 degrees celcius while normal fridge is 4 celcius). Would that affect the results of the test? Thanks!
ReplyDeleteDear Ron,
DeleteThe sample should still be useful.
Dr. Pheo
Dear Dr,
ReplyDeleteI have a question for you from spain. I contacted you last year. I had three tachycardia episodes and mild face hot feeling. I checked my 5HHIA.elevated wothout diet (13.2) and notrmal with diet (5.8). My new question is regarding my mum, 59 yo. She have had several episodes of tachycardia and elevated bp at same time. The episodes last around one hour. She has been refered to her endocrinologist for further investigations (blood work and urine 24h catecolamides). She had a mild flushing in her face and neck in the last episode. She has tyroid issues (enlarged tyroid nodule) and TSH or T4, i do not remember, in the low normal range. Do you think could pheo? carcinoid? have the carcionoid a genetic pattern? my episodes aare related to my mum's? Excuse my English. I wish you well. Regards
Pablo
Dear Anonymous,
DeleteIf menopausal symptoms are ruled out, she can easily test for pheo and carcinoid. Familial carcinoid is only reported in a single family so is exceedingly rare.
Dr. Pheo
Thanks Doctor. The doctors ruled out my carcinoied (although muy last urine 5-hiaa was 5.8 and normally i see mediana of 2.5 por 3...). I didnt have more flushing and palpitations in the last year. A sporadic diarrhea and constipation. Just only a mild hipertension in sporadic cases. My mothers have had hot intolerance in the last years. The menopausy was years ago...
DeleteBest reagards
My chromogramin A was 32 for a limit of 96.
DeleteDear Sir,
DeleteMy mother got her urine catecholamines.
epinephrine (adrenaline): normal
norepinephrine (noradrenaline): 110 (nr: <80)
dopamine 520 (nr: <400)
She has a cyst in the kidney.
No diet and she is anxyous. She is under beta blocker.
What do you think?
Thank you so much
Pablo
Dear Pablo,
DeleteThese values of urine catecholamines do not strongly suggest pheo. They more likely are false positive results, especially considering she is anxious.
Dr. Pheo
Dear Doctor,
DeleteThis is my last question.
My mother is waiting for her urine 24h metanephrines test.
I have begun having new episodes of hot feeling on my left cheek and neck. Also I have episodes of diarrea /loose stools and rectal bleeding (I have an hemorrhoid but I am feeling a prolapsed internal bump. Taking into account my 5-HIAA was 13.8 mg/d (without diet) and 5.8 mg/d with diet (0-7 nr, however I see means/medias of 3-3.5 in healthy people) and my mom's symptoms, do you suggest further evaluation or tests? Thank you Doctor
Pablo
Dear Pablo,
DeleteYou may want to measure chromogranin A and serotonin (markers of carcinoid).
Dr. Pheo
Dear Dr Pheo
ReplyDeleteI am a 60 year old woman. 16 months ago I began having episodes of very high blood pressure and tachycardia (200/130; hrt rate 140). Previously my blood pressure had always been low (90/60). During these episodes I would have full body shaking/shivering, headache, sweating, ringing in my ears, and bladder/bowel urgency, intense tingling in feet, legs into abdomen and arms, left upper abdominal pain.
A CT scan showed a 1 cm left adrenal nodule; 27 Hounsfield units pre-contrast; enhancing to 72 HU at 60 seconds; with a 36% washout after 15 minutes. 6 months later the adrenal nodule was still 1 cm but was 31 HU pre-contrast, enhanced to 111 HU at 60 seconds and had a 48% washout after 15 minutes.
From July 2014 I was having episodes 3-4 times a day (range 150/95 – 180/115) – mostly at night with severe episodes (200/130) approximately once a month. The episodes would last 15-60 minutes and often my BP would drop very low within a few minutes of onset (70/50 – 64/46). I have been unable to get a plasma metanephrine test done during an episode and so far all metanephrine tests have been in the normal range. One plasma metanephrine test was done 25 minutes after an episode and the normetanephrine was in the very high normal range. My Chromogranin A is 100 (normal is = or < than 100). The 24 hour urine catecholamine tests have also been normal. Thyroid blood tests all normal too. I’ve been in the Hospital Emergency Department 16 times in the last year due to severe episodes. Since these episodes began - CT contrast dye triggers high BP episodes. CT contrast dye did not have this effect prior to the onset of this condition.
I have been on Norvasc for 5 months now. On the Norvasc my blood pressure is usually 70/50 with severe breakthrough episodes every 3-4 weeks (range 180/110-200/138; hrt rate 130) and I have minor episodes daily. My doctors have ruled out anxiety attacks, P.O.T.S. and I have had normal head CT’s, chest x-rays & ECGs. I’ve been a meditator for 30+ years and until this began I was a daily runner and did weights. I have lost 10 lbs in the last 6 months. I am now 99 lbs. I have seen two cardiologists who both suspect that I have a pheo. An endocrinologist and an endocrine surgeon have indicated they will not diagnose me with pheo (or do surgery) until I get positive metanephrine blood results. I understand that with small pheos it can sometimes be difficult to get positive blood and urine results. I am scheduled to have another adrenal CT scan in 6 months. I have 2 questions:
1. Could this be a pheo - how would you suggest I proceed at this point?
2. Do you have any insight on the effect of a salbutamol inhaler (ventolin) on an adrenal nodule/pheo? (I have had asthma since infancy and have been on a salbutamol inhaler for 20 years - and other inhalers (isuprel) and medication (tedrol) beginning in early childhood. I stopped taking the salbutamol 2 months ago and the daily episodes have decreased. However, I still have episodes weekly and have had 2 severe episodes in the past two months.)
Thanks - JDK
Dear JDK,
DeleteOverall, a clinically significant pheo is unlikely. You could have a small pheo but small pheos usually do not cause symptoms. Asthma inhalers can cause hypertension and palpitation at high doses. I am not aware of any effects of inhalers on pheo per se.
Dr. Pheo
I have had increased Dopamine on 24 hour urine tests. Epinephrine and Norepinephrine are normal. I've had sudden onset of high blood pressure 170/ 124, heart rate only 65- 70. I can not breath when I have those onsets of high blood pressure. I've seen Endo 4 times in last few months. Two 24-hour urine tests were positive, one negative (Metanephrines Free 24 h 0.233 both times, Dopamine 24 h first time 3764 nmol/d. second time 3474 nmol/d). I am doing forth 24 h urine test next week. I have 1,5 cm visible adrenal adenomaon few MRI's. I do not drink smoke or use any kind of drugs. I have puffy ankles, like 3 golf balls arounf each ankle and gain about 25 lb in last 1,5 year. All other adrenal disorders except Pheochromocytoma are ruled out by my Endo. Kidneys, lungs, liver and heart are ruled out as causes of my high blood pressure. Any thoughts, suggestions? Thanks
ReplyDeleteDear Anna,
ReplyDeleteUrine dopamine levels are not a reliable marker of pheo. I recommend that you measure plasma catecholamines and metanephrines. Overall the likelihood of a clinically significant pheo is low.
Dr. Pheo
Thank you. Any other thoughts what can possibly be my diagnosis? As I mentioned I have had visible adrenal adenoma (found out accidently after my car accident in November 2011).
DeleteDear Anna,
DeleteI cannot think of anything obvious.
Dr. Pheo
Dr Pheo
ReplyDeleteThank you. I really appreciate your thoughts. I’d read your May 2012 blog re small pheos and appreciated your openness. In my case, my doctors seem to think that there may be something else triggering what might be a small pheo. My elimination of salbutamol has reduced my episodes to 2-3 a week – instead of several daily. So, if I have a pheo, ventolin may have been one trigger. I’m looking for what else might be a trigger. I use a pulmacort inhaler (a corticosteroid) for my asthma now – and I know in one of your blogs you’ve mentioned a possible link with corticosteroids and pheo episodes. I also have severe renal vein stenosis and high cholesterol. Have you ever heard of a small pheo triggering episodes of high blood pressure as in my case? Are you aware of other factors/triggers that might be involved in the case of a functioning small pheo?
My query about salbutamol and pheo was prompted by the cautions by pharmaceutical companies related to salbutamol/ventolin and pheo – “Some medical conditions may interact with Ventolin aerosol.” These lists include pheochromocytoma. I wondered if any research had been done on this link. Thanks again for the many insights in your blogs – very, very helpful! JDK
Dear JDK,
DeleteThanks for sharing the info.
Dr. Pheo
Dear Dr.Pheo,
ReplyDeleteHow do patients with succinate accumulation present clinically? Is there fluid accumulation in the body, or is it only a cellular event? Which eye abnormalities are more common with pheo? Are scintillating scotoma likelier with pituitary tumors, or are they part of pheo symptoms as well? What percentage of Cowden syndrome patients experience pheo? Is large head size a good enough clue for suspecting Cowden? When external pressure on the adrenal gland leads to bp spikes, shakiness, dizziness, difficulty breathing, headaches and blurred vision, do those symptoms point to pheo, or is there another more common diagnosis?
Thank you for your insight.
Dear Anonymous,
DeleteI don't know the answers to all the questions. On the other hand, if you want to know if you have pheo associated with SDH, you can simply measure plasma metanephrines. Your symptoms are indeed suggestive of pheo.
Dr. Pheo
Dear Dr. Pheo,
ReplyDeleteThank you for your blog, it has been very helpful. I am wondering if alcohol intolerance/allergy can be an indication of pheo or carcinoid. It is my understanding that aldehyde dehydrogenase deficiency can be responsible for alcohol intolerance, and that it is a mitochondrial component. What is the best test for that? Is aldehyde dehydrogenase deficiency related to any of the sdh deficiencies? What is the best test for sdh deficiencies? Do patients with Sjogren Larssen syndrome experience pheos, paras, and carcinoid at a higher rate than normal? What are the indications that increase suspicion of pheo, para, and carcinoid, and how are they differentiated?
Thank you for your assistance.
These are great questions. Unfortunately I don't know the answers.
DeleteDr. Pheo
Hi - What is the mechanism behind heat intolerance in pheo? Just curious.
ReplyDeleteThank you!
Heat intolerance is not common in pheo. It may be caused by higher metabolic rate.
DeleteDr. Pheo
Hi Dr. Pheo,
ReplyDeleteI was dx w/B9 Pheo in 2010. Had R adrenalectomy, its five years since srx. I have done f/u MRI annually. Imaging has been negative. I'm looking to find a specialist to help me with annual f/u. Dr. Manger who you have listed in your blog had retired. Is there anyone else you can recommend in NYC? TIA
You can ask Dr Manger himself or use the major medical centers in NYC, such as Columbia, Mount Sinai, Einstein, or Cornell.
DeleteDr. Pheo
Hi,
ReplyDeleteI contacted you early in the year reg my Mum.
She has a tumor (benign) on adrenal gland and had suspected Pheo. Her urine/bloods were negative though.
She has since seen an Endocronologist, and been tested for Carcinoid, Cushings, Aldosterone levels etc - All normal. The Endocronologist is not concerned with the lump.
Then she has seen a Gastroenterologist who basically just said based on symptoms, it could have been a virus! She did no tests.
She has now just been left to get on with it.
Her symptoms started as severe high BP (220 over 140) with light head, flushing, shaking.
She still gets quite high BP, and sometimes very low. She then started to get nausea, neck ache (not pain) has severe fatigue, and still gets the original symptoms.
Just now she had an 'episode' She is sitting down, and then goes light headed, palpatations, flushing, and shakes. It is 100% not anxiety. As she is 80, it is a big worry. It has gone on for 8 months now, and she had a few weeks of BP like this about two years ago also.
Based on the above, is their anything you can suggest or what the next step should be?
Many thanks, Samantha
Dear Samantha,
ReplyDeleteIt is a tough situation. I have more than a couple of elderly patients like that. I suggest a psychiatric evaluation.
Dr Pheo
I have been having these symptoms for over 9 years, I am a normally otherwise healthy 32 mom to two. I was induced with both pregnancy's because of high blood pressure. Have been extremely heat sensitive but after taking cymbalta for two days (for nerve pain) my episodes have gone from extreme activity and heat induced to almost anything, walking, sitting, sleeping, taking care of my kids can set it off and I get hot tingly from my lower abdomen up to my face and down my arms. Start with shakes and nausea and feeling as if I will either pass out or throw up.
ReplyDeleteI have been through 5 Primary dr's who all wrote it off as anxiety or stress only now have a found a Nurse Practitioner that literally stayed up all night one night to try to help me get answers ...... her answer was Pheo. . .. I only had my blood work done yesterday and am in the process of urine collection. .....
While I wouldn't wish this on anyone a big piece of my heart is happy to perhaps have a name to go along with this dibilitating symptoms that have been ruinning my life.
I am from Southern New Jersey, 20 minutes east of Philadelphia and am currently scouring the web trying to find any specialists near by so that if and when a diagnosis is made I can get appointments schedule ASAP
in the mean time I only have valium to take when an episode gets out of control, is there anything else I can do to help stop or deminish episodes
thanks so much
emily
emily magana at g mail dot com
I should also add that its been multiple trips to the ER thinking I was having a stroke, heart attack, feeling of impending doom
ReplyDeletehave been checked for lupus, limes, MS, you name it... they tested me for it and all have come back negative.....
Dear Anonymous,
ReplyDeleteLet's first see what the test results will be.
Dr. Pheo
Hello
ReplyDeleteI had/have an adrenal adenoma of 1.8 cm that had been followed from 2004 to 2010 with numerous suspiciously elevated 24 hour urine tests and a MIBG scan with some uptake. That finding was a "possible pheo". The entire matter was dropped upon Thyroid Cancer discovery in 2011. I had a Total thyroidectomy and was staged at stage 4 pap cancer but no recurrAnce. I recently had four trips to the ER and numerous months, weeks with heart palps, severe night sweats, feelings of doom, skin crawling of electrical sensations and goosebumps, feelings of hips and legs will give out, food sensitivity episodes from cheese/ sausage/ msg,Thai food, etc that make my heart rate and chest pound as well as my left side of my head , neck and ear pound and my left arm and armpit go limp. I had a 24 hour urine test in the hospital. All came back elevated. The adenoma is now 2cm according to the MRI I just had. The total metanephrine was 430pg/ml. Renin activity was 6.53 ng/ml/hr and Normetaneph was 402pg/ml. Aldosterone was 12ng/dl.
I was taking flexeril on and off for muscle pains and fibromyalgia pains. Then I wS given nortryptoline which made my recent symptoms worse and sent me to the ER. I had stopped taking the nortryptoline within two weeks of the prescription....god awful drug. I am no longer on flexeril either because it seems to become toxic to me. A pheo or not???
I also have a braff gene mutation and a hurthel cell mutation. My mom and sister also had pap thyroid cancer and my mom had a pituitary gland cystic tumor called a craniopharyngioma.
ReplyDeleteDear Elizabeth,
DeleteSorry for the late response. I need more information.
1. Your age
2. Did your mom receive radiation therapy for her craniopharyngioma? There is some link between radiation therapy and thyroid cancer.
3. What are the imaging characteristics of your adrenal mass, such as Hounsfield units, enhancement, etc?
4. What is the normal reference range for normetanephrine in your hospital?
Dr. Pheo
Thank you for responding, and for your continued interest.
Delete1. I am now 63. I was hypothyroid since age 36. .Thyroidectomy was 4.5 years ago. Cancer broke through Thyroid capsule. I had RAI treatment after surgery. No metatasis and no recurrence.
2. My Mom received pinpoint Radiation Therapy. The tumore burst upon removal. Surgeon said it was a gross mess. She takes Cortef every day. She is now 91 and in a nursing home. I believe her Pap Thyroid cancer came first. Then a few years later the craniopharyngioma was discovered.
3. The Adrenal mass I have had been described on MRI reports as an adenoma and was stable at 1.8 cm. It is now 2 cm. As previously stated, the matter was dropped, until now. My current symptoms are consistent with a pheo, including a trigger of awful symptoms if I eat cheese, bananas, sausage, or lie down in a prone position.(severe night sweats, heart pounding, weird whooshing feelings, weird back pain, neck throbbing, stomach muscle cramping, leg pain, tingling) My WBC goes up and down as does my RBC and protein count. I cannot use any drug that interferes with brain chemicals like Pamelor, buspirone or Flexeril. I get strange spine sensations, parasthesia in my extremities.
I have always suffered from severe night sweats, heart palps/chest pounding upon lying down but never even thought about the adrenal gland again after the thyroid cancer diagnosis. I normally do not see doctors unless it is my Thyroid checkup and mammogram.
3. In 2007,at that time, the MIBG scan showed "mild uptake in the region of the left adrenal gland of unclear significance, however, a pheocromocytoma is a possibility". I cannot find info on Hounsfeld units.
The MIBG in 2007 -Technique used: "10.2 mCi of I-131-labeled MIBG was administered intravenously without incident. Then, 24 hours, post injection, planar whole body images and SPECT images of ab and pelvis were obtained."
4. NOVEMBER 2015 URINE: Total Metanephrine was 430 pg/ml. Normal reference range of lab is <=205 pg/ml
Normetaneph was 402 pg/ml. Normal Lab ref range is <=148 pg/ml
Renin Activity was 12. Normal lab ref range is 0.25-5.82
Aldosterone- lab was unable to flag abnormal results for some reason.
I requested the 24 urine test as an inpatient. (11/13/15) I have been to the ER 5 times and admitted 3 of those times. I need to get back to the U of Penn?
Note: My blood pressure is normally 100/70. Withing the hospital ER and as an inpatient these last 3 months, it has sometimes gone as high as 138/112, 141/90. Unusual for me but it has not raised an eyebrow in the medical community. I had read that a functioning, small pheo need not have extreme Hypertension to be a ruled out as a pheo. As I have other symptoms consisent with one. Is this your opinion as well?
DeleteAlso (sorry for the lengthiness) a most disturbing symptom is severe muscle cramps, spasms, tightening and parasthesias and weakness in my lower trunk, legs and feet, tingling, waves of skin sensations. My new Primary doctors feels that a pheo can be causing all of this. Your opinion?
ReplyDeleteDear Elizabeth,
DeleteThe normal reference values you listed are for plasma metanephrines, not urine metanephrines. I assume you actually had plasma metanephrines tested. Small pheos (<3 cm) usually do not cause hypertension or clear symptoms except for pheo crisis. If the MRI clearly called your adrenal nodule an "adenoma", then you probably do not have pheo. Your situation is on the complex side. It will be better that you see a pheo specialist directly.
Dr. Pheo
I am wondering if my 83 year old mother has a pheo. She has arrhythmias accompanied by sweating that can last for days or weeks, then disappear. She reports that the tachycardia appeared at about the same time she was diagnosed with high blood pressure. I consider the high blood pressure significant because she has low plasma cortisol (.9 at 10 AM) and is on 20 mg cortef. She has chronically low plasma salt levels and I would expect low blood pressure rather than high.
ReplyDeleteBased on your blog, I'd like her to have a plasma metanephrines test but I wonder if her meds would make interpretation difficult: Toprol12.5 mg (beta blocker) Norvasc 5 mg, Lorazepam 2mg.
Quest Diagnostics says that beta blockers and sympathomimetics (Lorazepam?) can interfere. Do you recommend going ahead with the test, or tapering down and then being free of the drugs for 1 - 2 weeks prior to testing?
Dear Kathy,
DeleteI would just test metanephrines without changing the medications.
Dr. Pheo
Dr Pheo,
DeleteThanks very much for your recommendation and for this website. We will follow up with the metanephrine test.
Dr. Pheo,
ReplyDeleteI'm going back to the University of Penn Hospital to Dr. Fraker and Dr. D. Cohen. I've seen them before for this possible pheo. I was told Dr. Fraker (Endocrine surgeon may want to remove it due to its growth even though it is small. And, yes, those values were plasma, my error. I have been sent test orders to complete prior to my appointment.
Dr. Pheo,
ReplyDeleteI'm hoping to get your advice on my symptoms that have gotten worse over the past 6 years. My symptoms include orthostatic hypotension, periods of higher blood pressure but then back to normal, fatigue, low libido, depression, irritability & sometimes rage, constipation, abdominal pain/discomfort (thinking due to constipation), flushing, palpitations, shortness of breath, low avg body temp (97.5 and below generally), some sharp pains in one side of head but not frequent, hair loss primarily on calfs, geographic tongue, and occasional night sweats. I have aortic insufficiency, but not sure all these symptoms are caused by that. I've been told it's stress because most tests I have come back negative. Cortisol & DHEA are normal. Do you think this could be a pheo or something else?
Thank you,
KaseyDen
Dear KaseyDen,
ReplyDeleteThe best approach is to test your plasma metanephrines levels to check if you have pheo.
Dr. Pheo
Dr. Pheo,
ReplyDeleteI recently received my genetic test results from 23andme. I have the raw genotype data and was curious to see if I have the genetic markers for a pheo. Could you tell me what to look for in the raw data or point me to a resource that can?
Dear Brian,
DeleteTry this website: https://ghr.nlm.nih.gov/condition/hereditary-paraganglioma-pheochromocytoma#genes
Dr. Pheo