A few years ago, I wrote on the growth speed of pheo. Pheo,
contrary to conventional wisdom, grows slowly, at an average speed of one tenth
of an inch (0.2 cm) every year. Recently, a study showed that the biochemical
markers of pheo appear to also rise slowly. This study is interesting in a few
aspects. It was based on the Department of Defense Serum Repository (DoDSR).
The DoDSR is a large serum bank of all members of the US military forces and
boasts of 50 million specimens. The specimens were collected on annual physicals.
The authors used a smart research strategy. They identified people who had ever
served in the US military and been diagnosed with pheo. They then tracked down
the patients’ serum specimens and measured the metanephrine and normetanephrine
levels in the specimens. For example, a person could be enrolled in military
service at age 20 and diagnosed with pheo at age 40. All his serum specimens
since he (most of the subjects were men, as expected) joined the military were
available for testing. The authors actually picked only 3 specimens for testing.
The authors found out that the serum metanephrine or
normetanephrine levels began to be elevated years before diagnosis. For
example, the levels turned abnormal about 6 years before pheo diagnosis and
were 3-fold elevated about 4 years before diagnosis. The average time of
doubling of the marker levels was about 3 years. Individual patients had very
wide differences in the rise of marker levels. For example, some patients had a
doubling time less than 1 and half years, while some others had a doubling time
more than 8 years. The authors thus concluded that a steady and slow rise of
pheo marker levels is very suggestive of pheo. Because there were no imaging
data, we don’t know if the rise of pheo marker levels is related to tumor
growth, which is a limit of the study. Other studies do show parallel increase
in pheo marker levels and tumor burden AFTER diagnosis.
Dr. Pheo
Hi again Dr Pheo
ReplyDeleteI received my genetic test results today and was told I had an inconclusive variant on my VHL gene. I had a left sided Pheo at age 30 (now 38)and no other VHL symptoms to date although Ive never been screened for any VHL symptoms. The genetic counselor said my particular variant has been recorded one other time in a person who did in fact have clinically significant VHL. She said this and some of my family history indicates that I should have some VHL screening done. It was also suggested that my children may need to have the VHL genetic tests as a result of this inconclusive. My question is have you heard of other cases where inconclusive variants in VHL gene actually ended up full blown VHL? Is it reasonable to proceed with the VHL screening and testing the children? As always, your input is greatly appreciated.
J
Dear J,
DeleteIt is not uncommon to find a variant with unknown significance. There is quite a bit of controversy and debate over this issue. No simple right or wrong answer. If your insurance overs the screening or you can afford it, I would screen yourself for potential other VHL tumors and your children for that variant.
Dr. Pheo
Hi Dr. Pheo. I was diagnosed with a retroperitoneal tumor. I've been to several doctors and repeat scans. The tumor hasn't changed size in over a year and a half. It is 5.4 cm. The 24 hour Urine test came back fine. They have it differential as paraganglioma, ganglioneuroma, schwannoma, or neurofibroma. I have no high blood pressure, no head aches, and no other symptoms. A recent doctor suggested a biopsy. I'm getting a repeat 24 hour urine test and blood test done first, but my question to you is can a biochemically silent paraganglioma become active during a biopsy?
ReplyDeleteDear Unknown,
DeleteBiopsy should be safe if the urine test for pheo returned normal results.
Dr. Pheo
Thank you!!
DeleteDr. Pheo, I have been having symptoms off and on for many years, main symptoms are tachycardia, high blood pressure but goes up and down, burning sensations on my skin that is transient, headaches, irritability, weight loss without trying All of the symtoms are episodic. I can also be very sensitive to heat, but I dont sweat so I tend to overheat easily. I have also recently been diagnosed with primary hyper parathyroid and also have a multi nodual goiter with well over 20 nodules on both sides of my thyroid. Thyroid funtions are normal, no indication of it being the cause of the tachy. I have been to the ER 3 times due to tachy, had echo and holter 24 hours on day with no symptoms. All have come out fine. My Endo has ordered the 24 hour urine test however catching a day where I have symptoms in the morning and can start the test is really difficult. My endo is recomending removal of thyroid and parathyroid tumor. I am not sure if I need the thyroid removed and am nervous about surgery without having ruled out the pheo. Is there any other test that is easier than the 24 hour urin to rule this out prior to surgery? There have been no other identified causes for my symptoms. P.S. I posted this same question on another topic but took your advice to post on the most recent. Thank you!
ReplyDeleteOne more thought, my Endo does not believe my calcium levels, although slightly elevated, are high enough to cause my symptoms. Hence suspicion of Pheo. However, when I was in the ER for sypmtoms in October of last year my phosphate levels were extremely low and I had to have a 7 hour drip.
DeleteDear Anonymous,
DeleteHigh calcium and low phosphate levels are consistent with hyperparathyroidism.
Of note, hyperparathyroidism and pheo are both components of multiple endocrine neoplasia syndrome type 2.
Dr. Pheo
Final note based on some of your questions to other posts. Test is for catacholamines and was told to wait till I had symptoms, the tricky part. I am a 43 year old female. I also have periods of frequent night waking where I then can not go back to sleep with elevated heart rate. Most unusual are the 3 episodes where i have woken up with uncontrollable shivering. In the winter it seemed odd but maybe I was that cold.. however, it just recently happened again and we do not make our house very cool in the summer at all. This episode brought my concernt to a new level. Thank you again!!
ReplyDeleteThe plasma metanephrines test is the best.
DeleteDr. Pheo
I am glad I found you blog Dr. PHEO and I hope you might give me your opinion on my case. I am a mostly healthy 41YO mother of 4. Several months after the birth of my 2nd child in 2003, I started having episodes of high blood pressure, flushing, extreme sweating, palpitations, and tremors. I had always been intolerant to heat, but had never had any of the other symptoms. My only other issues at the time were frequent headaches and chronic lumbar and thoracic pain. The endo said I had early Graves disease, nonessential tremors and woulI am glad I found you blog Dr. PHEO and I hope you might give me your opinion on my case. I am a mostly healthy 41YO mother of 4. Several month after the birth of my 2nd child, I started having episodes of high blood pressure, flushing, extreme sweating, palpitations, and tremors. I had always been intolerant to heat, but had never had any of the other symptoms. My only other issues at the time we're frequent headaches and chronic lumbar and thoracic pain. The endo said I had early Graves disease, nonessential tremors and would need to keep a check on my thyroid and gave me a beta blocker of the tremor. Several years went by and I went to work at a larger hospital. The sweating, flushing, and tremor got worse. The other symptoms were not as frequent. Over the next few years all of the symptoms got worse. I also hurt my back @ the gym and had back surgery that did seem to work. The sweating got so bad I was always drenched at work and flushed. We lived in coastal SC, so it is very humid, but I was sweating no matter what the season. I had to go on disablity because of my back, without work I could hide at home as much as possible because I was and still am so embarrassed to be so sweaty. Over the next couple of years my blood pressure started to creep up to 140-145/85-90 and I was diagnosed with hypertension. It was frequently normal at home. During this time I would stay up for 2 or 3 days unable to sleep even with ambien. I ended up in the hospital for 5 days because my pulse shot up to 160, my blood pressure was 160-170/120, and I had the worst headache of my life. They thought I had meningitis. The did a spinal tap and head ct, but found nothing. My discharge papers said anxiety. Over the last few years the sweating has gotten even worsen, my normal pulse is 80-85, but several times a day it is above 100. My blood pressure still fluctuates wildly. My hands tremor, it sometimes feels like my arms are shaking and my teeth are chattering. I have also become very fearful over the last 13 years. My family would describe me a having a sense of dome.
ReplyDeleteAfter reading about Pheochromcytoma, I ask my PCP to run the plasma metanephrine labs, knowing it was a long shot.
RESULTS
Metanephrine <25 range <=57
Normetanephrine <412 range <=148
Would you recommend more labs and/or an abdominal CT. Should I try to find a specialist in Georgia or South Carolina.
Thanks
Dear L.B.,
ReplyDeleteYou can repeat the test in 3-6 months. If the results are in the same range, then abdominal CT is a reasonable next step.
Dr. Pheo
Dr. Pheo,
ReplyDeleteI am writing simply because I am hoping for a little guidance. I have a 20 year old son that is currently on swallowed Budesonide due to Eosinophilic Esophagitis, and has a severe allergy to peanuts. In the last year he has had a rash that tends to come on when gets warm and his heart rate increases. The doctor originally thought that it was cholinergic urticaria, but then decided to run some urine test. His first VMA came back high at 23.6mg/24h (range 6 or less). Then the doctor started mentioning Pheo. They then did a Metanephrine, HVA, and Creatinine and they all came back in normal range. They repeated the VMA and it also came back in normal range. His blood IgE is very high, and his Dopamine low. Also, somewhat low bone density on the Dexa scan. I realize these things don't have to do with Pheo, but want to give you a clear picture. I have read on the likelihood of false positives in urine VMA, but really just want to be sure that further investigation isn't necessary. Any advice would be greatly appreciated!!! Thanks for your time.
Dear Mom,
ReplyDeleteYour son unlikely have pheo.
Dr. Pheo
Hi Dr. My son is a healthy 17 year old that is 5'11" and 188 lbs. He is very muscular and active. He has begun to present with high blood pressure w/ spikes, excessive sweating and headaches. His primary care has given him a full workup including liver ultrasound due to elevated liver enzymes. This came back normal.
ReplyDeleteHis cardiologist has ruled out cardiac reasons for the high blood pressure. He did order a lot of tests, all which came back normal except his 24 hour urine test. The results are as follows:
Epinephrine, U, 24 hr - 22 (0-18)
Norepinephrine, U, 24 hr - 124 (0-90)
Dopamine, Ur, 24 hr - 618 (0-575)
Normetanephrine, U, 24 hr - 454 (63-402)
(Hypertensive <18 years (110-1050))
Metanephrine, U, 24 hr - 330 (32-167)
(Hypertensive <18 years (35-460))
He has had high blood pressure and headaches for several months, but the sweating began about six weeks ago. His cardiologist put him on liscinopril and referred us to an endocrinologist. We are going Friday, but after research and reading your blog, I am thinking this doctor will probably not have had experience with pheos. We are in the Atlanta area and thinking we should probably go to Emory University Hospital. Are familiar with any doctors there that are pheo-knowledgeable? Also, what do you think of my son's symptons/tests thus far? My son will be 18 in October. Thank you.
This comment has been removed by the author.
DeleteDr., additional symptom my son has shown is tremors when he gets upset. What are the next step tests we should request when going to the endocrinologist?
DeleteDear Susan,
DeleteEmory University or National Institute of Health in Bethesda, Maryland will be great choice. Abdominal imaging is likely needed.
Dr. Pheo
Thanks so much!
DeleteHi Dr. Pheo,
DeleteWe are still trying to get a diagnosis. The doctor has ordered a few blood tests including the plasma metanephrines (we are 10 days and still waiting on the results). She did order a CT scan of the abdominal area and it was clear. She is going to schedule an MIBG scan when the results are back. My question to you is your thoughts on the MIBG vs. FDOPA - PET scan?
She is looking for an extra-adrenal Pheo/Para but I am not confident in her experience in this. My goal is to get the diagnosis and then find an experienced doctor at Emory or even the NICH.
My last question for you today; what is your thoughts on someone going to the National Institute of Children's Health for treatment?
I will be researching tomorrow for a doctor for diagnosis if I continue to feel uneasy with this doctor. Thank you very much!
Dear Susan,
DeleteFDOPA is little better than MIBG. Dr Karel Pacak at the National Institute of Health is the utmost expert on pheo, especially in children.
Dr. Pheo
Good day Dr. Pheo,
ReplyDeletefirst of all nice blog and your support means a lot.
After doing a fundoplication 6 years ago I started suffering from the following deseases: obesity, hypertension, sleep apnea, diverticulitis, IBS, sinus tachycardia.
Few weeks ago my cardiologist ordered me a 24 urine analysis and seems that my hormones came very high so he ordered me an urgent MRI. Lately have been suffering from severe headaches at the back of my head, an my BP has been pumping up, having sometimes my left eye red and hissing in my ears. The headaches start when I cough or sneeze and remain there for a day and literally have to go off from work.
MRI result though came normal except for small cysts in kidney and liver and diverticulitis.
Have visited an endocrinologist now but he said he will check my results and revert but nothing yet.
My surgeon ordered me an MRI of the skull and he is stating that if it comes normal than he does not know what I can do and maybe it is uncontrollable hypertension.
Any suggestions ?
Thanks
Dear Godfrey,
DeleteCan you write out the exact hormones tested, results, and normal ranges?
Dr. Pheo
Dont have the actual results but think it was VMA and came 10 times as much
ReplyDeleteHigh VMA in 24-hr urine 87.1 on 5/6/2016. Normal 5-HIAA
ReplyDeleteDear Godfrey,
ReplyDeleteIn that case, you do need to check the retroperitoneal space, in and around blader, and the chest (around the heart) for paraganglimomas.
Dr. Pheo
My chest x ray shows enlarge heart. Meanwhile did another MRI head but came normal too. Waiting for results of methanephrines.
DeleteHi,
ReplyDeleteSorry if my English is not so good. I’m 38 years old, and I have around two years having some symptoms. They started with episodes of nervousness, pale skin, strange felling in the belly a little tickling in the back of the head, sweating, etc.., this episodes last like 5 minutes, and after that everything was normal. I had them like twice a day, sometimes more. Sometime after I found that this episodes came with bradycardia (around 45 bpm) I was felling palpitations and felt the slow rate, so I started to measure the heart rate. And after some time I measured my blood pressure during an episode and it was around 150/95, after the episode my BP returned to normal levels like 110/70. So I decided to go to a cardiologist, he didn’t know exactly what I had and started to give me medicine for hypertension because he measured the BP and it was like 150/90, but at home I measured it and it was normal all the times. I was very stressed at that time, so I decided to quit the medicine (because I was almost sure I wasn’t hypertensive) and to change habits to see if that change something.
Few weeks ago, this episodes become more frequent and some are coming with very severe headaches. I tried to measure my BP in some of that episodes (not the strongest ones) and my BP was around 180/110 and my HR around 38 bpm (maybe a reflex bradycardia), after the episodes the BP goes around 110/70 and HR 60 to 80 bpm. I went to another cardiologist, first he thought I had a vasovagal reflex. But after some days the headaches became stronger and unbearable so he told me to do a 24 urine test looking for metanephrines, and the results showed the normetanephrines in 2400, so he think I could have a pheochromocytoma, today I’m going for a CT scan and the results will be on Monday. I’m very scared.
I live in Mexico City, I will ask my cardiologist if he knows a specialist here in Mexico, but I’m having thoughts to go to USA to address this problem, but I don’t know where to start. Can you give me any suggestions for a good point to start if I do it in USA?
Thanks in advance.
Regards
Dear Anonymous,
DeletePlease check a post in 2009 about pheo specialists in the world. There are several pheo specialists in US.
Dr. Pheo
Hi Dr. Pheo,
ReplyDeleteI recently happened on your blog while trying to find some information. I have been having quite a few symptoms over the course of a few years which seem to be getting worse. Symptoms such as exercise intolerance, headaches, sweating, angry outburst and stomach pains. Recently I have begun to notice that the symptoms seem worse after I eat. After a few years of complaining and adjusting my thyroid medication, my doctor finally listened to me. During some of the lab work she also ordered a thoracic MRI for another reason. The radiologist found a mass lesion near the left adrenal gland measuring around 3cm. She recommended a follow up CT scan which my doctor ordered but when it came back they didn't mention the adrenal glands at all except to say that there were no renal masses found in either kidney. My physician is sending me for the lab work to rule out possible phao and another CT scan. Is it possible for a mass to show up on the MRI but not on the CT scan?
Dear Flower,
ReplyDeleteCT and MRI should see the same adrenal lesion. You should ask your doctor about the adrenal lesion on CT scan.
Dr. Pheo
Is there any adice you can give before taking the 24 hour urine test, like if there is something I shouldn't take or eat before or during? My allergist wants me to take it to check for pheo and my histamine level. I am 35 and have had a wacky history of tachycardia (even after having an ablation for an AVNRT), hypertension, and blood sugar levels since I was 18 (that I know of), despite using ateninal and hydrochlorithiozide in the past and on toprol xl now and despite keeping my sodium intake below 1000-1500 on average. Before my ablation, my SVTs were just below 300 bpm and now my tachycardia is between 110-150 bpm. My normal (before my insane initial spike a few years ago) bp is about 110/60 and now it can jump up to 185/120 (which is where it was when it sent me to the ER and when I found out about my hbp to begin with). My bp fluctuations happen almost instantly. Earlier, it was 147/97 and within 5 minutes it dropped down to 102/71. I could check it an hour later and it will be like 178/110 and I hadn't done anything that would be expected to raise it and it would give me a major headache like a migraine or sinus headache. Is my allergist possibily over reacting (her nurse saw I was taching when she took my vitals the other day and freaked out...I had to explain my history to her nurse to calm her down, she got so scared)?
ReplyDeleteThis comment has been removed by the author.
ReplyDeleteDear VampDarling,
DeleteI suggest that you first do a plasma metanephrines test instead (without any precautions).
Dr. Pheo
Thank you. I will discuss doing that with my allergist. I have to say that it would be nice to have a simple quick fix solution for it all, but I'm not going to dwell on the possibility of it and do not want to add another surgery to my medical history.
ReplyDeleteI went ahead and did the 24hr urine test because I had already started it and my results just came back. I don't know much about it, but here they are:
ReplyDeleteMetanephrine:
162 (52-341) ug 24hr
Normetanephrine:
454 (88-444) ug 24hr
Total Metanephrines:
616 (140-785) ug 24hr
Histamine:
329 nmol/L
Creatinine:
65 mg/dL
Histamine nol/g Creat:
506 nmol/L Cr
Ref. Range:
0-450 nmol/L Cr
I went ahead and did the 24hr urine test because I had already started it and my results just came back. I don't know much about it, but here they are:
ReplyDeleteMetanephrine:
162 (52-341) ug 24hr
Normetanephrine:
454 (88-444) ug 24hr
Total Metanephrines:
616 (140-785) ug 24hr
Histamine:
329 nmol/L
Creatinine:
65 mg/dL
Histamine nol/g Creat:
506 nmol/L Cr
Ref. Range:
0-450 nmol/L Cr
Dear VampDarling,
DeleteThe test results make pheo unlikely.
Dr. Pheo
Thank you for creating this blog. It's been very helpful reading all the posts and the comments. I'm wondering how likely it is that I might have a pheocromocytoma. I've been going to several doctors over the past couple of months since becoming ill and most are very confused. I am 3 months postpartum and was in the hospital recently with sepsis. The source of the infection wasn't found. Abdominal CT was clear. Doctors told me I was a medical mystery. Since being in the hospital I developed tachycardia. My symptoms are similar to pots. My bp drops to 70/50 after breastfeeding but has been borderline high since delivery. My fatigue is so bad that I need help doing regular activities. The 24 hr test is the only lab that has numbers out of range. Dopamine was 525 range is 52-480. VMA is 9.4 range is 6 or less. E+NE 113 range 26-121.norepinephrine 24 hr 100 range 15-100. Epinephrine 13 range 2-24. My doctor told me he hasn't had a patient with elevated levels and ordered an migb scan to look for a pheocromocytoma. He told me he doesn't have experience with this and is referring me to an Endocrinologist. I was reading that results are usually 3 fold with pheocromocytomas.
ReplyDeleteDear Niki,
DeletePheo is unlikely. Consulting a pheo specialist of course will help.
Dr. Pheo
Thank you. I was able to see a specialist on the list and he was very helpful. He too does not think I have a pheochromocytoma and does not recommend an mibg scan. Especially since my abdominal MRI and CT were clear. I did have the following blood tests done:
DeleteMetanephrine Free <25 pg/mL < OR = 57 pg/mL
Normetanephrine, Free 131 pg/mL < OR = 148 pg/mL
Total, Free (MN + NMN) 131 pg/mL < OR = 205 pg/mL
ACTH, Plasma 6 ng/L 6 - 50 ng/L
Chromogranin A <5 ng/mL
< OR = 15 ng/mL
Plasma Renin Activity 0.95 ng/mL/h 0.25 - 5.82 ng/mL/h
I talked to my primary doctor about my results and the recommendation of the specialist. My primary doctor still thinks I should do the scan due to my tachycardia and 24 hr urine results. I am confused and worried about the exposure to the radioactive tracer and would prefer not to do the scan. After reading your blog post I don't think I am one of those rare patients. I've never had a pheo and it doesn't run in my family. Is there a large risk with doing the mibg scan? I've read online I would need to stay away from others for 48 hours after the test but the hospital staff told me it's less radiation than ct and X-ray. The tech also said I would not expose those close to me with any radiation. What would you do? The more information I have the more confused I become.
Dear Niki,
ReplyDeleteThere is no need to do the MIBG scan.
Dr. Pheo
Hi Doc,
ReplyDeleteI am hoping that I am correct in the reassurance this finding has given me. I tested Plasma and 24hr Ur (not because one led to the other, my GP and Endo ordered different tests at the same time without knowing), both were negative. This was in July 2017. The reason was infrequent episodes of anxiety, palpitations, and BP spikes.
I had some reprieve, but I got two bad ones that sent me to the ER on two separate occasions in January a couple of weeks apart. With these, My HR and BP were higher than the previous episodes (120s hr - 180/100 BP). These tended to subside once I got to the hospital. I had everything worked up again (cardio/endo/GP), by my doctors who are chalking it up to panic disorder at this point (I am an anxious person, and these episodes came after months of insomnia and stress).
The only test I didn't retake was the pheo tests. If I took them in July (6 months prior to the episodes), are my doctors right in not retesting so soon?
Dear Nervous,
ReplyDeletePheo is ruled out for you already. No need to do another test.
Dr. Pheo